Materiały źródłowe

• #1 Erythrocytosis (Polycythaemia): Definition, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23468-erythrocytosis

  Erythrocytosis causes you to have high levels of hematocrit and/or hemoglobin. […] Too many red blood cells can impact your body in a variety of ways depending on whats causing your erythrocytosis. Some effects are mild while others are more serious. Many people only experience unpleasant symptoms, like headache or fatigue. More serious causes of erythrocytosis can cause your blood to become too thick, putting you at risk of blood clots, heart attacks or strokes. […] Your symptoms (including how severe they are) will depend on whats causing your erythrocytosis. For example, secondary causes of erythrocytosis may cause symptoms that include headaches, confusion, trouble sleeping, weakness and fatigue. Primary causes of erythrocytosis may cause more severe symptoms, including high blood pressure, night sweats, unexplained weight loss, severe pain, swelling and tenderness in your joints (gout), bleeding problems, including frequent nosebleeds and bruising easily, itchy skin that may feel worse after youve been in warm water, tingling (pins and needles) feeling in your arms, legs, hands or feet, burning and redness, especially in your face, hands or feet. […] Your experience depends on whats causing your erythrocytosis. Many inherited causes of primary erythrocytosis only cause mild symptoms and arent life-threatening. More serious conditions, like polycythemia vera, require lifelong treatment.

• #1 Stages of polycythemia vera: Early, final, and more

  https://www.medicalnewstoday.com/articles/stages-of-polycythemia-vera

  Polycythemia vera (PV) is a rare disorder that affects blood cell and platelet production. The early stages of polycythemia vera often cause nonspecific symptoms, but advanced stages can lead to myelofibrosis and other severe complications. […] Symptoms of PV tend to develop gradually over years. […] In the early stages, the condition may cause no apparent symptoms. A healthcare professional may diagnose it following a routine blood test before any symptoms develop. […] As time passes, more noticeable symptoms may occur. The disease may also cause complications, such as an enlarged spleen, chest pain, a heart attack, or a stroke. […] A person may not have noticeable symptoms in the early stages of PV. Any symptoms that appear are often nonspecific. […] For example, early symptoms may include fatigue, weakness, dizziness, headache, unusual sweating, particularly at night, itchy skin, which may get worse after a warm bath or shower.

• #1 Erythrocytosis: What Is It, What Causes It, and More

  https://www.webmd.com/a-to-z-guides/what-is-erythrocytosis

  Many of the symptoms of erythrocytosis are caused by your blood flowing more slowly than normal. These symptoms can include: […] Blurred vision […] Headaches […] Confusion […] High blood pressure […] Nosebleeds […] Itching […] Weakness […] Tiredness.

• #1 Erythrocytosis

  https://www.nhs.uk/conditions/erythrocytosis/

  Erythrocytosis, sometimes called polycythaemia, means having a high concentration of red blood cells in your blood. […] Many of the symptoms of erythrocytosis are caused by this sluggish flow of blood. […] Not everyone with erythrocytosis has symptoms but many do. […] Make an appointment to see your GP if you have persistent symptoms of erythrocytosis. These include: headaches, blurred vision, red skin, particularly in the face, hands and feet this may be more difficult to see on black or brown skin, tiredness, high blood pressure, dizziness, discomfort in the tummy, confusion, bleeding problems such as nosebleeds and bruising, gout which can cause joint pain, stiffness and swelling, itchy skin especially after a bath or shower. […] Erythrocytosis can cause blood clots. […] Erythrocytosis also increases your risk of heart attack and stroke.

• #1 Polycythemia vera Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/polycythemia-vera

  Polycythemia vera (PV) is a bone marrow disease that leads to an abnormal increase in the number of blood cells. The red blood cells are the most affected blood cell type. […] In people with PV, there are too many red blood cells in the blood. This results in thicker blood, which can’t flow through small blood vessels normally, leading to symptoms such as: Trouble breathing when lying down, Dizziness, Feeling tired all the time, Full feeling in the left upper abdomen (due to enlarged spleen), Headache, Itchiness, especially after a warm bath, Red skin coloring, especially of the face, Shortness of breath, Symptoms of blood clots in veins near the skin surface (phlebitis), Vision problems, Ringing in the ears (tinnitus), Joint pain. […] PV usually develops slowly. Most people do not have symptoms related to the disease at the time of diagnosis. The condition is often diagnosed before severe symptoms occur.

• #1 Erythrocytosis: Definition, Causes, and Symptoms

  https://www.healthline.com/health/erythrocytosis

  Symptoms of erythrocytosis include: headaches, dizziness, shortness of breath, nosebleeds, increased blood pressure, blurred vision, itching. […] Having too many RBCs can also increase your risk for blood clots. If a clot becomes lodged in an artery or vein, it can block blood flow to essential organs like your heart or brain. A blockage in blood flow can lead to a heart attack or stroke. […] Often the conditions that cause erythrocytosis can’t be cured. Without treatment, erythrocytosis can increase your risk for blood clots, heart attack, and stroke. It can also increase your risk for leukemia and other types of blood cancers. […] Getting treatment that lowers the number of RBCs your body produces can reduce your symptoms and prevent complications.

• #1 Polycythaemia | nidirect

  https://www.nidirect.gov.uk/conditions/polycythaemia

  Polycythaemia, or erythrocytosis, means having a high concentration of red blood cells in your blood. You should also get emergency medical help if you think you or someone you’re with is having a heart attack or stroke. Many of the symptoms of polycythaemia are caused by this sluggish flow of blood. Mild cases of polycythaemia may not cause any problems, but some people with polycythaemia can experience: discomfort in the chest or tummy (abdomen), muscle ache and weakness, tiredness, headaches – may be experienced as a sense of 'fullness’ in the head and neck, dizziness, tinnitus – hearing sounds that come from inside your head, when there is no sound externally, blurred vision (or temporary loss of vision in one or both eyes), paraesthesia – an abnormal sensation such as tingling, tickling, pricking, numbness or burning, with no clear physical cause, periods of confusion, bleeding problems such as nosebleeds, heavy periods and bruising, itchy skin especially after a bath or shower – this is the result of white blood cells (levels of which can also be high) releasing the chemical histamine, red skin particularly in the fingers, palms, heels or toes, gout which can cause joint pain, stiffness and swelling. You should make an appointment to see your GP if you have persistent symptoms of polycythaemia. The slow blood flow associated with polycythaemia can also cause blood clots. These can be serious because they may put you at risk of life-threatening problems such as heart attacks, pulmonary embolisms a blockage in the blood vessel that carries blood from the heart to the lungs, strokes. Signs of DVT or a pulmonary embolism can include: pain, swelling, redness and tenderness in one of your legs, a heavy ache in the affected area, warm skin in the area of the clot, breathlessness, chest or upper back pain, coughing up blood, feeling lightheaded or dizzy, fainting. If you experience any of the above symptoms, seek medical help immediately. Many cases are mild and may not lead to any further complications. Some cases particularly cases of PV can be more serious and require long-term treatment. If well controlled, polycythaemia shouldn’t affect your life expectancy, and you should be able to live a normal life. People with PV can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes. PV can also sometimes cause scarring of the bone marrow (myelofibrosis). This can eventually lead to you having too few blood cells. In some rare cases, the condition can develop into a type of cancer called acute myeloid leukaemia (AML). If you have polycythaemia, it’s important to take any medication you’re prescribed. It is also important to keep an eye out for signs of possible blood clots to help reduce your risk of serious complications.

• #1 Polycythemia vera – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/polycythemia-vera/symptoms-causes/syc-20355850

  Many people with polycythemia vera don’t notice symptoms. Some people get symptoms such as headache, dizziness, tiredness and blurred vision. […] Clearer symptoms of polycythemia vera include: Itchiness, mostly after a warm bath or shower. Numbness, tingling, burning or weakness in the hands, feet, arms or legs. A feeling of fullness soon after eating. Bloating or pain in the left upper stomach area due to an enlarged spleen. Unusual bleeding, such as a nosebleed or bleeding gums. Painful swelling of one joint, often the big toe. Shortness of breath and trouble breathing when lying down. Bone pain. […] Without treatment, polycythemia vera can be life-threatening. But proper medical care can help ease symptoms and complications of this disease.

• #1 Investigation and management of erythrocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7829024/

  Primary erythrocytosis or autonomous production of excess erythrocytes most commonly occurs due to polycythemia vera (PV), a myeloproliferative neoplastic process that may be asymptomatic or may present with thrombosis, constitutional or vasomotor symptoms, or splenomegaly. […] Patients with PV may have symptoms of splenomegaly, constitutional symptoms or vasomotor symptoms such as headache, visual disturbances or light-headedness. Two specific symptoms for myeloproliferative neoplasms are pruritus and erythromelalgia. Pruritus is often aquagenic and may be debilitating. Erythromelalgia is a recurrent burning sensation accompanied by erythema and warmth, most commonly affecting the hands. […] The goals of treatment of PV are to reduce the risk of arterial and venous thromboembolism, and minimize symptoms. Unfortunately, existing treatments do not successfully reduce the risk of transformation to myelofibrosis or acute leukemia.

• #1 Secondary Erythrocytosis – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/myeloproliferative-disorders/secondary-erythrocytosis

  Secondary erythrocytosis is erythrocytosis that develops secondary to disorders that cause tissue hypoxia, inappropriately increased erythropoietin production, or increased sensitivity to erythropoietin. […] Any elevation of hemoglobin or hematocrit above normal values for age and sex is considered erythrocytosis. […] Patients with chronic hypoxemia (arterial hemoglobin oxygen concentration 92%), typically due to lung disease, right-to-left intracardiac shunts, renal transplantation, prolonged exposure to high altitudes, or hypoventilation syndromes, often develop erythrocytosis. The primary treatment is to alleviate the underlying condition, but oxygen therapy may help, and phlebotomy may decrease viscosity and alleviate symptoms. […] In some people, phlebotomy is used to lower the number of red blood cells, but phlebotomy is rarely needed in secondary erythrocytosis.

• #1 Secondary polycythemia: Its Symptoms, Diagnosis, Treatment, and Management

  https://www.webmd.com/a-to-z-guides/what-is-secondary-polycythemia

  Secondary polycythemia, also known as secondary erythrocytosis or secondary erythrocythemia, is a rare condition in which your body produces an excess amount of red blood cells. […] This thickened blood cant pass through your small blood vessels like capillaries easily. This increases your risk of having a stroke. […] Patients with secondary polycythemia may have symptoms including: Fatigue, Headache, Dizziness, Blurred vision, Anorexia, Weakness, Reduced mental acuity. […] Secondary polycythemia symptoms occur because of the thickening of your blood after red blood cells are overproduced; in some cases, the overproduction is accompanied by a decrease in the volume of plasma. […] Depending on the underlying cause (what caused secondary polycythemia), your symptoms may vary.

• #1 Secondary Polycythemia: Causes, Treatment, Symptoms, and More

  https://www.healthline.com/health/blood-cell-disorders/secondary-polycythemia

  Symptoms of secondary polycythemia include: breathing difficulty, chest and abdominal pain, fatigue, weakness and muscle pain, headache, ringing in ears (tinnitus), blurred vision, burning or pins and needles sensation in hands, arms, legs, or feet, mental sluggishness. […] Secondary polycythemia (erythrocytosis) is a rare condition that causes your blood to thicken and increases the risk of stroke. […] If the polycythemia makes the blood extremely viscous, there’s an increased risk of stroke. […] Secondary polycythemia doesn’t always require treatment. When needed, treatment is usually low-dose aspirin or blood drawing (phlebotomy).

• #1 Erythrocytosis

  https://www.nhs.uk/conditions/erythrocytosis/

  Treatment for erythrocytosis aims to prevent symptoms and complications (such as blood clots), and treat any underlying causes. […] Some people may also need treatment for any other symptoms or complications of erythrocytosis they have, or for any underlying cause of the condition. […] The outlook for erythrocytosis largely depends on the underlying cause. […] Many cases are mild and may not lead to any further complications. However, some cases particularly cases of polycythaemia vera can be more serious and require long-term treatment. […] If well controlled, erythrocytosis should not affect your life expectancy, and you should be able to live a normal life. However, people with polycythaemia vera can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes.

• #1

  https://111.wales.nhs.uk/encyclopaedia/e/article/erythrocytosis

  Erythrocytosis, sometimes called polycythaemia, means having a high concentration of red blood cells in your blood. […] This makes the blood thicker and less able to travel through blood vessels and organs. Many of the symptoms of erythrocytosis are caused by this sluggish flow of blood. […] Not everyone with erythrocytosis has symptoms but many do. […] Make an appointment to see your GP if you have persistent symptoms of erythrocytosis. These include: headaches, blurred vision, red skin particularly in the face, hands and feet, tiredness, high blood pressure, dizziness, discomfort in the tummy, confusion, bleeding problems such as nosebleeds and bruising, gout which can cause joint pain, stiffness and swelling, itchy skin especially after a bath or shower. […] Erythrocytosis can cause blood clots. These put you at risk of life-threatening problems such as: pulmonary embolisms a blockage in the blood vessel that carries blood from the heart to the lungs, deep vein thrombosis (DVT) a blockage that forms in the blood vessels in your leg before moving elsewhere in your body.

• #1

  https://111.wales.nhs.uk/encyclopaedia/e/article/erythrocytosis

  Erythrocytosis also increases your risk of heart attack and stroke. Seek emergency medical help if you think that you or someone you’re with is having a heart attack or stroke. […] The outlook for erythrocytosis largely depends on the underlying cause. […] Many cases are mild and may not lead to any further complications. However, some cases particularly cases of polycythaemia vera can be more serious and require long-term treatment. […] If well controlled, erythrocytosis should not affect your life expectancy, and you should be able to live a normal life. However, people with polycythaemia vera can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes. […] Polycythaemia vera can also sometimes cause scarring of the bone marrow (myelofibrosis), which can eventually lead to you having too few blood cells. In some rare cases, the condition can develop into a type of cancer called acute myeloid leukaemia (AML).

• #1 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  Fatigue, insomnia, and difficulty concentrating are the most common presenting symptoms in patients with PV. […] Poor prognostic features include age older than 60 years, history of thrombosis, leukocytosis, high JAK2 burden, abnormal karyotype, and established cardiovascular risk factors such as smoking, hypertension, diabetes, obesity, and hyperlipidemia. […] Without treatment, death typically occurs within two years, mostly from thrombotic events. […] Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. […] For those diagnosed before 60 years of age, median survival is 24 years. […] The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. […] Progression to myelofibrosis over 15 years is 6% to 14%.

• #1 Polycythemia: Definition, causes, symptoms, and more

  https://www.medicalnewstoday.com/articles/polycythemia

  It is not uncommon for a person to have polycythemia and be unaware of it. Symptoms tend to build very slowly over time. […] Having too many red blood cells thickens the blood, which makes healthy blood flow more difficult. It can also increase a persons risk of blood clots. This risk is especially high if a person with PV has an excess of both red blood cells and platelets. […] The symptoms of PV may become more apparent over time. More common symptoms include: dizziness or vertigo, headaches, excessive sweating, itchy skin, ringing in the ears, blurred vision, fatigue, reddish or purplish skin on the palms, earlobes, and nose, bleeding or bruising, a burning sensation in the feet, abdominal fullness, frequent nosebleeds, bleeding gums. […] Without treatment, people with PV may also be more likely to experience complications, such as: enlarged spleen, blood clots, angina, stroke, peptic ulcers, heart disease, gout, other blood disorders, such as myelofibrosis or leukemia.

• #1 Polycythaemia vera | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/polycythaemia-vera

  Polycythaemia vera is characterised by the production of too many red blood cells. […] Typically, the onset of disease is sometime after the age of 50 years, with a slow but persistent progression. […] The symptoms of polycythaemia vera include: Headache, Dizzy spells, Itching skin, especially after bathing, Flushing of the skin, especially the face, Breathlessness, Phlebitis (inflammation of the vein), Vision problems, Skin rashes, Blue tinge to the skin (cyanosis), Fatigue, Gout, Kidney stones, Enlarged spleen (splenomegaly). […] Without treatment, around half of all people with symptomatic polycythaemia vera will die in less than two years. There is no cure, but treatment can extend the person’s life span by thinning the blood and reducing the risk of blood clots and other complications.

• #1 High Red Blood Cell Count (Polycythemia): Symptoms & Treatment

  https://www.emedicinehealth.com/polycythemia_high_red_blood_cell_count/article_em.htm

  Symptoms of polycythemia vera can be vague and quite general such as headache, easy bleeding, and more. […] Symptoms of polycythemia vera can be vague and quite general. Some of the most significant symptoms include: Easy bruising, Easy bleeding, Blood clot formation (potentially leading to heart attacks, strokes, and blood clots in the lungs [pulmonary embolism]), Bone and joint pain (hip pain or rib pain), Headache, Itching, Itching after taking a shower or bath (post-bath pruritus), Fatigue, Dizziness, Abdominal pain. […] People with primary polycythemia need to be aware of some of the potentially serious complications that may occur. The formation of blood clots (heart attacks, strokes, blood clots in the lungs [pulmonary embolism] or legs [deep vein thrombosis]) and uncontrolled hemorrhaging (nosebleeds, gastrointestinal bleeding) typically require prompt medical attention by the treating physician or emergency department. […] The outlook for primary polycythemia without treatment is generally poor, with a life expectancy of about 2 years. However, with phlebotomy alone, many patients lead normal lives and enjoy a normal life expectancy.

• #1 Polycythemia Vera : Symptoms, Causes, Stages, Diagnosis and Treatment

  https://oncodaily.com/oncolibrary/cancer-types/225800

  Early symptoms of polycythemia vera are often subtle and non-specific, making timely diagnosis challenging. Weakness, shortness of breath, and blurred vision are common early indicators of PV. Weakness results from impaired oxygen delivery despite the increased red blood cell count, while shortness of breath may occur during physical exertion due to heightened blood viscosity. Blurred vision is often caused by reduced blood flow to the eyes. […] These vague symptoms are frequently misattributed to less serious conditions, leading to delays in diagnosis. […] In the proliferative phase, there is a marked increase in red blood cell production, often accompanied by elevated white blood cells and platelets. Symptoms such as fatigue, headaches, dizziness, itching, and an enlarged spleen (splenomegaly) are common. Thrombotic events, including strokes and deep vein thrombosis, become more likely due to increased blood viscosity.

• #1 Polycythemia Vera : Symptoms, Causes, Stages, Diagnosis and Treatment

  https://oncodaily.com/oncolibrary/cancer-types/225800

  In a subset of patients, PV can progress to myelofibrosis, a condition characterized by bone marrow scarring (fibrosis). This leads to reduced blood cell production (cytopenias), severe anemia, and splenomegaly. Symptoms include extreme fatigue, weight loss, and night sweats. […] In rare cases, PV can evolve into AML, a highly aggressive cancer of the blood and bone marrow. This transformation is associated with poor prognosis and limited treatment options, often requiring intensive chemotherapy or experimental therapies.

• #1 Secondary Polycythemia: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/205039-overview

  In secondary polycythemia, the number of red blood cells (RBCs) is increased as a result of an underlying condition. […] Secondary polycythemia most often develops as a response to chronic hypoxemia, which triggers increased production of erythropoietin by the kidneys. […] To the extent that the increased RBCs alleviate tissue hypoxia, secondary polycythemia may in fact be beneficial. However, treatment with phlebotomy is indicated for patients with hematocrits higher than 60%-65%, who may experience symptoms such as impaired alertness, dizziness, headaches, and compromised exercise tolerance, and who may face increased risk for thrombosis, strokes, myocardial infarction, and deep venous thrombosis. […] At extreme levels of secondary polycythemia, patients can be at risk for thrombosis. Excessive polycythemia, usually defined as hematocrit levels higher than 65-70%, may result in increased whole blood viscosity. This, in turn, may lead to impaired blood flow locally, resulting in thrombosis. […] Hyperviscosity may also lead to generalized sluggish blood flow, resulting in impaired tissue oxygenation in multiple organs, which may lead to decreased mentation, fatigue, generalized weakness, and poor exercise tolerance.

• #1 Reddit – The heart of the internet

  https://www.reddit.com/r/polycythemiavera/comments/11evflu/primarysecondary_polycythemia_symptoms_and/

  In general, my HCT levels have been between 48-52 and my HGB levels have been between 16 -19.5 in the past two years. […] headaches […] HGB: Migraines appear at 16.5 +, debilitating migraines at 18.0+ […] blurred vision […] HGB: 19.4+ […] red skin particularly in the face, hands, and feet […] HGB: 16.5 ish+ […] tiredness […] HGB: feeling tired at 16.0 ish+, fatigued at 16.5+, exhausted and need bed rest at 18.0+ […] high blood pressure […] HGB: 18.0 […] dizziness […] HGB: mild dizziness at 16.5 ish+, high-level dizzy at 18.0+ […] confusion […] HGB: low levels at 16.5 ish+, high levels at 18.0+ish […] itchy skin especially after a bath or shower […] HGB: 16.0 ish+ […] tinnitus […] HGB: 16.0ish+ […] While all of the above symptoms can come and go independently of each other, sometimes many at one time, this and fatigue have been the only consistent symptoms that begin before all other symptoms and disappear as soon as I receive phlebotomy.

• #1 Erythrocytosis due to presumed polycythemia vera | MDedge

  https://community.the-hospitalist.org/content/erythrocytosis-due-presumed-polycythemia-vera

  A 40-year-old woman with hypertrophic obstructive cardiomyopathy presents to the hematology clinic for a second opinion regarding a history of headaches and fatigue for the past 10 years. She has been diagnosed with idiopathic erythrocytosis, presumed to be due to polycythemia vera. She periodically undergoes phlebotomy to keep her hematocrit below 41%, and this markedly improves her headaches. […] Symptoms arise from an increase in the total blood volume and red blood cell mass, often leading to dilated capillaries and other blood vessels. Symptoms can occur regardless of the cause and classically include headache (often described as diffuse heaviness), dizziness, and a tendency for bleeding or thrombosis. Symptoms are relieved when the hematocrit is lowered. […] Symptoms such as severe itching after exposure to hot water (aquagenic pruritus) and periodic attacks of redness, swelling, and pain in the hands or feet, or both (erythromelalgia), have been described in patients with polycythemia vera. […] Compared with patients with polycythemia vera, patients with high-oxygen-affinity hemoglobin have fewer complications from hyperviscosity and thrombosis, even with comparable degrees of erythrocytosis.

• #1 Diagnosing or Ruling Out Polycythemia Vera in Patients With Erythrocytosis – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/january-2019/diagnosing-or-ruling-out-polycythemia-vera-in-patients-with-erythrocytosis/

  The symptoms are hugely variable, depending on the cause. Erythrocytosis may cause no symptoms at all, or it may be highly symptomatic and detrimental to health. […] When they do occur they are quite specific, and include aquagenic pruritus, erythromelalgia, symptoms of arterial or venous thromboses, and gout. In addition, the risk for transformation to myelofibrosis in patients who have polycythemia vera is approximately 15%; in such cases, they present with fatigue, bone pain, sweating, and symptoms of splenomegaly, such as early satiety and/or splenic pain. The risk for transformation to acute leukemia is lower, at 3% to 5%, in which case the symptoms are the same as those in any acute leukemia. […] Symptoms of other polycythemias/erythrocytoses are nonspecific, with most patients who have an increased number of red cells experiencing no symptoms. Rare patients may have symptoms such as fatigue and headaches resulting from hyperviscosity; these symptoms should resolve with phlebotomy.

• #1 Polycythemia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK526081/

  Polycythemia, also called erythrocytosis, refers to increased red blood cell mass, noted on laboratory evaluation as increased hemoglobin and hematocrit levels. […] The clinical significance of erythrocytosis, due to any cause, is related to the associated risk of thrombotic events due to hyperviscosity of blood. Additionally, in cases of polycythemia vera, there is potential for progression to leukemia. […] The clinical significance of erythrocytosis, due to any cause, lies in the associated risk of thrombotic events due to hyperviscosity of blood. Additionally, the potential for progression to leukemia in cases of polycythemia vera also warrants additional management strategies to be implemented. […] Progression to leukemia, particularly acute myeloid leukemia (AML), is seen in approximately 5% of cases and is often refractory to treatment. Studies have implicated the use of chlorambucil, pipobroman, or radioactive phosphorous as factors that increase the likelihood of progression.

• #1 Secondary Erythrocytosis – Blood Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/blood-disorders/myeloproliferative-disorders/secondary-erythrocytosis

  Erythrocytosis is increased production of red blood cells (erythrocytes). […] People with secondary erythrocytosis may have weakness, tiredness, headache, light-headedness, and shortness of breath.

• #1 Diagnosing or Ruling Out Polycythemia Vera in Patients With Erythrocytosis – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/january-2019/diagnosing-or-ruling-out-polycythemia-vera-in-patients-with-erythrocytosis/

  Blood clots are the major complication in patients with polycythemia vera, and blood clots are even more common in those with Chuvash polycythemia. Common dogma dictates that a high hematocrit is the cause of blood thickening and blood clots, but I am skeptical that this is the major cause. No evidence exists that a high hematocrit is harmful and a direct cause of thrombosis; in addition, many conditions that lead to a very high hematocrit are not associated with thromboses.

• #1 Investigation and management of erythrocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7829024/

  Secondary erythrocytosis can be distinguished from PV in most patients with a focused clinical evaluation and, where available, determination of the erythropoietin level and JAK2 V617F mutation testing. Goals of treatment in PV are to alleviate symptoms, reduce the risk of thromboembolism, and monitor patients for transformation to myelofibrosis or acute leukemia. The majority of patients with PV should be treated with low-dose ASA and phlebotomy to achieve a target hematocrit value of less than 0.45. Cytoreduction, most commonly with hydroxyurea, should be considered in patients at high risk for thrombosis. Treatment of secondary erythrocytosis should be directed at the underlying cause.

• #1 Polycythemia Vera in Children | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/p/polycythemia-vera-in-children.html

  Some children do not have symptoms, and polycythemia vera may be found when they’re having blood tests for other reasons. […] Symptoms can occur a bit differently in each child. They can include: […] The symptoms of polycythemia can be like other health conditions. Make sure your child sees their healthcare provider for a diagnosis. […] Complications depend on how severe the polycythemia is. They can include: […] Your child will be watched for changes in blood test results or signs and symptoms of complications. […] Call your child’s healthcare provider if you notice new or worse symptoms. For example, call if your child is more tired than usual.

• #1 Polycythaemia (Rubra) Vera – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/types-of-blood-cancer/myeloproliferative-neoplasms/polycythaemia-rubra-vera/

  A prognosis is an estimate of the likely course of a disease. […] In some people, polycythaemia progresses over time despite treatment. The spleen may become increasingly enlarged. Anaemia and thrombocytopenia (low numbers of circulating platelets) is common as the bone marrow is no longer able to produce adequate numbers of red cells or platelets. […] Treatment during this time is supportive and involves making every effort to improve the patient’s quality of life, by relieving any symptoms they might have and by preventing and treating any complications that arise from their disease or its treatment.

• #1 Polycythaemia vera (PV) symptoms | Blood Cancer UK

  https://bloodcancer.org.uk/understanding-blood-cancer/polycythaemia-vera-pv/pv-symptoms/

  Having blood thats too thick can cause blood to clot too easily. This is known as thrombosis. This can be serious, so its important to know what to look out for. The symptoms of thrombosis will vary depending on where the clot is, but they include: slurred speech, weakness on one side of the body, drooping on one side of your face, sudden chest pain, dizziness and feeling short of breath, one leg or arm becoming painful, swollen and warm, coughing up blood, sudden blurred vision or loss of sight, sudden pain in the stomach area, possibly with swelling and signs of jaundice (a yellowing of the eyes, and yellowish skin in people with lighter skin tones).

• #1 Polycythemia | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/polycythemia

  Infants and children with polycythemia often have no visible symptoms. When they do appear, the most common symptoms include: […] Deep reddish-purple coloring […] Poor feeding […] Tiredness or fatigue […] Shortness of breath or breathing difficulty […] Low blood sugar […] Headache […] Dizziness […] Itchiness, especially following a warm bath or shower […] Numbness, tingling, burning, or weakness in your hands, feet, arms, or legs.

• #1 Polycythemia Vera in Children

  https://www.nationwidechildrens.org/conditions/health-library/polycythemia-vera-in-children

  Some children do not have symptoms, and polycythemia vera may be found when they’re having blood tests for other reasons. […] Symptoms can be different in each child. They can include: […] Headaches. […] Dizziness. […] Feeling weak and tired. […] Trouble breathing. […] Enlarged spleen. […] Trouble seeing. […] Itching or burning. […] Reddish color of the face. […] Bleeding gums. […] Losing weight. […] The symptoms of polycythemia vera can be like other health conditions. Make sure your child sees their health care provider for a diagnosis. […] Problems depend on how bad the condition is. They can include: […] Deep vein thromboses or blood clots in the veins. […] Blood clots in the veins of the liver. […] Heavy bleeding. […] Stroke. […] Leukemia.

• #1 Familial erythrocytosis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/familial-erythrocytosis/

  Familial erythrocytosis is an inherited condition characterized by an increased number of red blood cells (erythrocytes). Signs and symptoms of familial erythrocytosis can include headaches, dizziness, nosebleeds, and shortness of breath. The excess red blood cells also increase the risk of developing abnormal blood clots that can block the flow of blood through arteries and veins. If these clots restrict blood flow to essential organs and tissues (particularly the heart, lungs, or brain), they can cause life-threatening complications such as a heart attack or stroke. However, many people with familial erythrocytosis experience only mild signs and symptoms or never have any problems related to their extra red blood cells. […] Familial erythrocytosis can have different inheritance patterns depending on the gene involved.

• #1 REVE2 – Overview: Erythrocytosis Evaluation, Blood

  https://www.mayocliniclabs.com/test-catalog/overview/618830

  The most common cause of hereditary erythrocytosis is the presence of a high-oxygen-affinity (HOA) hemoglobin variant. A subset of hemoglobins with increased oxygen (O2) affinity results in clinically evident erythrocytosis caused by decreased O2 unloading at the tissue level. Many are asymptomatic; however, some patients have recurrent headaches, dizziness, fatigue, and restless legs. A subset of patients experience thrombotic episodes. Affected individuals can be plethoric, and many are misclassified as polycythemia vera, particularly prior to more recent genetic testing availability. […] In addition, O2-sensing pathway variants, EPAS1(HIF2A) (OMIM 611783); EGLN1(PHD2) (OMIM 609820), and VHL (OMIM 263400) cause hereditary erythrocytosis and a subset are associated with pheochromocytoma and paragangliomas. All have shown an autosomal dominant pattern of inheritance, except VHL-associated erythrocytosis, which is an autosomal recessive disorder.

• #1 Case Study: 25-Year-Old Woman Referred to Clinic for Erythrocytosis – Hematology.org

  https://www.hematology.org/education/trainees/fellows/case-studies/25yo-woman-erythrocytosis

  A 25-year-old woman of Indian ethnicity was referred to hematology clinic for erythrocytosis. […] The patient noted a history of discoloration in her hands, as well as chronic fatigue. […] Her nail beds and lips showed a bluish discoloration. […] This vignette represents a case of autosomal recessive Type 1 methemoglobinemia. […] Methemoglobin is formed when heme Fe2+ is oxidized to Fe3+, which leads to a decreased ability to reversibly bind; thus, deficiencies in cytb5r can cause buildup of methemoglobin and inability to effectively deliver oxygen to tissues. […] As opposed to the more severe and often fatal type 2 methemoglobinemia, this form of methemoglobinemia is often only associated with cyanosis. […] Pulse oximetry will often show a low SpO2 despite normal SaO2 due to optical interference from high levels of methemoglobin.

• #1 Outcome of patient with Polycythemia Rubra Vera and psychiatric symptoms | Hematology, Transfusion and Cell Therapy

  https://www.htct.com.br/en-outcome-patient-with-polycythemia-rubra-articulo-S2531137920301036

  Polycythemia Vera (PV) is a chronic myeloproliferative neoplasia generating an accumulation of erythrocytes in the peripheral blood (polyglobulia). The disease is described as polysymptomatic and its typical manifestations consist of headache, dizziness, weakness, plethora, itching and splenomegaly, the last three being more suggestive. Thus, a diagnosis of polycythemia may be suspect when it cannot be explained by a secondary cause. […] Neuropsychiatric symptoms are described in the literature but are less prevalent in current clinical practice. […] The pathophysiology of psychiatric events in PV is not well established, but the proposed models consist of two mechanisms resulting from blood hyperviscosity: (1) slowed blood flow with hypoxia and (2) multiple thrombosis, small and disseminated by the Central Nervous System (CNS).

• #1 Reddit – The heart of the internet

  https://www.reddit.com/r/polycythemiavera/comments/11evflu/primarysecondary_polycythemia_symptoms_and/

  I discovered it was supposedly caused by nocturnal hypoxia, which was supposedly caused by Obstructive Sleep Apnea, which I also didn’t know I had. […] Despite my OSA having been somewhat resolved with CPAP, my secondary polycythemia-related symptoms have still been consistent, which has impacted my life through and through and has made my case a long-standing question mark. […] Following my oxygen levels on my Apple watch, I have observed that my oxygen levels still dip low at night (75%-88%, meaning the nocturnal hypoxia didn’t go away), and so I still believe my symptoms could be related to this or something I have missed. […] In fact, I question whether or not this could be possible. […] Essentially, I found this piece of information surprising since I think there could be other unknown overlapping problem(s), and so I think it would be important and helpful to ask/share hemoglobin/hematocrit values with others diagnosed with polycythemia to get a sense of which symptoms present and when.

• #1 Erythrocytosis: Definition, Causes, Symptoms, and More

  https://resources.healthgrades.com/right-care/blood-conditions/erythrocytosis

  Treatment centers on alleviating the cause of oxygen deprivation. Phlebotomy is sometimes used. Some causes of erythrocytosis, such as polycythemia vera, can be serious and require lifelong treatment. Treatment for erythrocytosis is essential to lower your risk of heart attacks, blood clots, and stroke.

• #2 Erythrocytosis

  https://www.nhs.uk/conditions/erythrocytosis/

  Erythrocytosis, sometimes called polycythaemia, means having a high concentration of red blood cells in your blood. […] Many of the symptoms of erythrocytosis are caused by this sluggish flow of blood. […] Not everyone with erythrocytosis has symptoms but many do. […] Make an appointment to see your GP if you have persistent symptoms of erythrocytosis. These include: headaches, blurred vision, red skin, particularly in the face, hands and feet this may be more difficult to see on black or brown skin, tiredness, high blood pressure, dizziness, discomfort in the tummy, confusion, bleeding problems such as nosebleeds and bruising, gout which can cause joint pain, stiffness and swelling, itchy skin especially after a bath or shower. […] Erythrocytosis can cause blood clots. […] Erythrocytosis also increases your risk of heart attack and stroke.

• #2 https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/signs-and-symptoms

  https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/signs-and-symptoms

  Polycythemia vera (PV) develops slowly, and it may not cause symptoms for many years. The condition is often diagnosed during a routine blood test before severe symptoms occur. […] Symptoms may include: […] When PV causes symptoms, they can be troublesome. Reducing symptoms is a key goal of treatment. Therefore, it is important to take an active role in monitoring your PV symptoms. Careful tracking of your symptoms can help you and your doctor better understand how to manage and modify your care over time.

• #2 Erythrocytosis (Polycythaemia): Definition, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23468-erythrocytosis

  Erythrocytosis causes you to have high levels of hematocrit and/or hemoglobin. […] Too many red blood cells can impact your body in a variety of ways depending on whats causing your erythrocytosis. Some effects are mild while others are more serious. Many people only experience unpleasant symptoms, like headache or fatigue. More serious causes of erythrocytosis can cause your blood to become too thick, putting you at risk of blood clots, heart attacks or strokes. […] Your symptoms (including how severe they are) will depend on whats causing your erythrocytosis. For example, secondary causes of erythrocytosis may cause symptoms that include headaches, confusion, trouble sleeping, weakness and fatigue. Primary causes of erythrocytosis may cause more severe symptoms, including high blood pressure, night sweats, unexplained weight loss, severe pain, swelling and tenderness in your joints (gout), bleeding problems, including frequent nosebleeds and bruising easily, itchy skin that may feel worse after youve been in warm water, tingling (pins and needles) feeling in your arms, legs, hands or feet, burning and redness, especially in your face, hands or feet. […] Your experience depends on whats causing your erythrocytosis. Many inherited causes of primary erythrocytosis only cause mild symptoms and arent life-threatening. More serious conditions, like polycythemia vera, require lifelong treatment.

• #2 Secondary Erythrocytosis – Blood Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/blood-disorders/myeloproliferative-disorders/secondary-erythrocytosis

  Erythrocytosis is increased production of red blood cells (erythrocytes). […] People with secondary erythrocytosis may have weakness, tiredness, headache, light-headedness, and shortness of breath.

• #2 Polycythaemia | nidirect

  https://www.nidirect.gov.uk/conditions/polycythaemia

  Polycythaemia, or erythrocytosis, means having a high concentration of red blood cells in your blood. You should also get emergency medical help if you think you or someone you’re with is having a heart attack or stroke. Many of the symptoms of polycythaemia are caused by this sluggish flow of blood. Mild cases of polycythaemia may not cause any problems, but some people with polycythaemia can experience: discomfort in the chest or tummy (abdomen), muscle ache and weakness, tiredness, headaches – may be experienced as a sense of 'fullness’ in the head and neck, dizziness, tinnitus – hearing sounds that come from inside your head, when there is no sound externally, blurred vision (or temporary loss of vision in one or both eyes), paraesthesia – an abnormal sensation such as tingling, tickling, pricking, numbness or burning, with no clear physical cause, periods of confusion, bleeding problems such as nosebleeds, heavy periods and bruising, itchy skin especially after a bath or shower – this is the result of white blood cells (levels of which can also be high) releasing the chemical histamine, red skin particularly in the fingers, palms, heels or toes, gout which can cause joint pain, stiffness and swelling. You should make an appointment to see your GP if you have persistent symptoms of polycythaemia. The slow blood flow associated with polycythaemia can also cause blood clots. These can be serious because they may put you at risk of life-threatening problems such as heart attacks, pulmonary embolisms a blockage in the blood vessel that carries blood from the heart to the lungs, strokes. Signs of DVT or a pulmonary embolism can include: pain, swelling, redness and tenderness in one of your legs, a heavy ache in the affected area, warm skin in the area of the clot, breathlessness, chest or upper back pain, coughing up blood, feeling lightheaded or dizzy, fainting. If you experience any of the above symptoms, seek medical help immediately. Many cases are mild and may not lead to any further complications. Some cases particularly cases of PV can be more serious and require long-term treatment. If well controlled, polycythaemia shouldn’t affect your life expectancy, and you should be able to live a normal life. People with PV can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes. PV can also sometimes cause scarring of the bone marrow (myelofibrosis). This can eventually lead to you having too few blood cells. In some rare cases, the condition can develop into a type of cancer called acute myeloid leukaemia (AML). If you have polycythaemia, it’s important to take any medication you’re prescribed. It is also important to keep an eye out for signs of possible blood clots to help reduce your risk of serious complications.

• #2 Polycythemia vera Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/polycythemia-vera

  Polycythemia vera (PV) is a bone marrow disease that leads to an abnormal increase in the number of blood cells. The red blood cells are the most affected blood cell type. […] In people with PV, there are too many red blood cells in the blood. This results in thicker blood, which can’t flow through small blood vessels normally, leading to symptoms such as: Trouble breathing when lying down, Dizziness, Feeling tired all the time, Full feeling in the left upper abdomen (due to enlarged spleen), Headache, Itchiness, especially after a warm bath, Red skin coloring, especially of the face, Shortness of breath, Symptoms of blood clots in veins near the skin surface (phlebitis), Vision problems, Ringing in the ears (tinnitus), Joint pain. […] PV usually develops slowly. Most people do not have symptoms related to the disease at the time of diagnosis. The condition is often diagnosed before severe symptoms occur.

• #2 Secondary Polycythemia: Causes, Treatment, Symptoms, and More

  https://www.healthline.com/health/blood-cell-disorders/secondary-polycythemia

  Symptoms of secondary polycythemia include: breathing difficulty, chest and abdominal pain, fatigue, weakness and muscle pain, headache, ringing in ears (tinnitus), blurred vision, burning or pins and needles sensation in hands, arms, legs, or feet, mental sluggishness. […] Secondary polycythemia (erythrocytosis) is a rare condition that causes your blood to thicken and increases the risk of stroke. […] If the polycythemia makes the blood extremely viscous, there’s an increased risk of stroke. […] Secondary polycythemia doesn’t always require treatment. When needed, treatment is usually low-dose aspirin or blood drawing (phlebotomy).

• #2

  https://111.wales.nhs.uk/encyclopaedia/e/article/erythrocytosis

  Erythrocytosis, sometimes called polycythaemia, means having a high concentration of red blood cells in your blood. […] This makes the blood thicker and less able to travel through blood vessels and organs. Many of the symptoms of erythrocytosis are caused by this sluggish flow of blood. […] Not everyone with erythrocytosis has symptoms but many do. […] Make an appointment to see your GP if you have persistent symptoms of erythrocytosis. These include: headaches, blurred vision, red skin particularly in the face, hands and feet, tiredness, high blood pressure, dizziness, discomfort in the tummy, confusion, bleeding problems such as nosebleeds and bruising, gout which can cause joint pain, stiffness and swelling, itchy skin especially after a bath or shower. […] Erythrocytosis can cause blood clots. These put you at risk of life-threatening problems such as: pulmonary embolisms a blockage in the blood vessel that carries blood from the heart to the lungs, deep vein thrombosis (DVT) a blockage that forms in the blood vessels in your leg before moving elsewhere in your body.

• #2 Polycythemia vera – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/polycythemia-vera/symptoms-causes/syc-20355850

  Many people with polycythemia vera don’t notice symptoms. Some people get symptoms such as headache, dizziness, tiredness and blurred vision. […] Clearer symptoms of polycythemia vera include: Itchiness, mostly after a warm bath or shower. Numbness, tingling, burning or weakness in the hands, feet, arms or legs. A feeling of fullness soon after eating. Bloating or pain in the left upper stomach area due to an enlarged spleen. Unusual bleeding, such as a nosebleed or bleeding gums. Painful swelling of one joint, often the big toe. Shortness of breath and trouble breathing when lying down. Bone pain. […] Without treatment, polycythemia vera can be life-threatening. But proper medical care can help ease symptoms and complications of this disease.

• #2 Polycythaemia Rubra Vera (High Red Blood Cell Count)

  https://patient.info/allergies-blood-immune/polycythaemia-rubra-vera-high-red-blood-cell-count

  Polycythaemia rubra vera (PRV) is a myeloproliferative disorder, which means the bone marrow makes too many blood cells. In PRV there is an abnormally high number of red blood cells in your blood. This is because the myeloid stem cells in your bone marrow have started to multiply out of control. As these stem cells can develop into either red blood cells, platelets or white blood cells, if you have PRV, you may also have an abnormally high number of platelets and white blood cells. […] An abnormally high number of red blood cells is called erythrocytosis. […] The sluggish blood flow means that oxygen (that is carried bound to haemoglobin in your red blood cells) cannot get to the tissues of your body as easily. This can lead to various symptoms, including: Headache. Tiredness. Dizziness. Ringing in your ears (tinnitus). Blurring of your vision. Chest pain or pain in your calf muscles when walking.

• #2 Erythrocytosis: Definition, Causes, and Symptoms

  https://www.healthline.com/health/erythrocytosis

  Symptoms of erythrocytosis include: headaches, dizziness, shortness of breath, nosebleeds, increased blood pressure, blurred vision, itching. […] Having too many RBCs can also increase your risk for blood clots. If a clot becomes lodged in an artery or vein, it can block blood flow to essential organs like your heart or brain. A blockage in blood flow can lead to a heart attack or stroke. […] Often the conditions that cause erythrocytosis can’t be cured. Without treatment, erythrocytosis can increase your risk for blood clots, heart attack, and stroke. It can also increase your risk for leukemia and other types of blood cancers. […] Getting treatment that lowers the number of RBCs your body produces can reduce your symptoms and prevent complications.

• #2 Stages of polycythemia vera: Early, final, and more

  https://www.medicalnewstoday.com/articles/stages-of-polycythemia-vera

  Polycythemia vera (PV) is a rare disorder that affects blood cell and platelet production. The early stages of polycythemia vera often cause nonspecific symptoms, but advanced stages can lead to myelofibrosis and other severe complications. […] Symptoms of PV tend to develop gradually over years. […] In the early stages, the condition may cause no apparent symptoms. A healthcare professional may diagnose it following a routine blood test before any symptoms develop. […] As time passes, more noticeable symptoms may occur. The disease may also cause complications, such as an enlarged spleen, chest pain, a heart attack, or a stroke. […] A person may not have noticeable symptoms in the early stages of PV. Any symptoms that appear are often nonspecific. […] For example, early symptoms may include fatigue, weakness, dizziness, headache, unusual sweating, particularly at night, itchy skin, which may get worse after a warm bath or shower.

• #2 Stages of polycythemia vera: Early, final, and more

  https://www.medicalnewstoday.com/articles/stages-of-polycythemia-vera

  Also, people with PV have an increased risk of blood clots, which may develop even in the early stages. They can cause potentially life threatening complications, such as a stroke or heart attack. […] Over time, some people with PV may develop MF. This occurs if scar tissue replaces bone marrow. When a person has MF, the bone marrow cannot produce enough healthy blood cells. […] Some symptoms of MF include fatigue, weakness, shortness of breath, unexplained weight loss, easily bruising or bleeding, fever, unusual sweating, particularly at night, pain or a feeling of fullness below the ribs on the left side. […] PV causes the blood to become thicker than usual. The blood clots more easily and may not flow through the blood vessels properly. As a result, it may carry less oxygen to the body’s tissues and organs. […] Over time, these changes can cause noticeable symptoms and potentially life threatening complications. […] Early treatment is important for relieving symptoms and reducing the risk of complications. Getting treatment may help improve a person’s quality of life and increase life expectancy.

• #2 Investigation and management of erythrocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7829024/

  Primary erythrocytosis or autonomous production of excess erythrocytes most commonly occurs due to polycythemia vera (PV), a myeloproliferative neoplastic process that may be asymptomatic or may present with thrombosis, constitutional or vasomotor symptoms, or splenomegaly. […] Patients with PV may have symptoms of splenomegaly, constitutional symptoms or vasomotor symptoms such as headache, visual disturbances or light-headedness. Two specific symptoms for myeloproliferative neoplasms are pruritus and erythromelalgia. Pruritus is often aquagenic and may be debilitating. Erythromelalgia is a recurrent burning sensation accompanied by erythema and warmth, most commonly affecting the hands. […] The goals of treatment of PV are to reduce the risk of arterial and venous thromboembolism, and minimize symptoms. Unfortunately, existing treatments do not successfully reduce the risk of transformation to myelofibrosis or acute leukemia.

• #2 Diagnosing or Ruling Out Polycythemia Vera in Patients With Erythrocytosis – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/january-2019/diagnosing-or-ruling-out-polycythemia-vera-in-patients-with-erythrocytosis/

  The symptoms are hugely variable, depending on the cause. Erythrocytosis may cause no symptoms at all, or it may be highly symptomatic and detrimental to health. […] When they do occur they are quite specific, and include aquagenic pruritus, erythromelalgia, symptoms of arterial or venous thromboses, and gout. In addition, the risk for transformation to myelofibrosis in patients who have polycythemia vera is approximately 15%; in such cases, they present with fatigue, bone pain, sweating, and symptoms of splenomegaly, such as early satiety and/or splenic pain. The risk for transformation to acute leukemia is lower, at 3% to 5%, in which case the symptoms are the same as those in any acute leukemia. […] Symptoms of other polycythemias/erythrocytoses are nonspecific, with most patients who have an increased number of red cells experiencing no symptoms. Rare patients may have symptoms such as fatigue and headaches resulting from hyperviscosity; these symptoms should resolve with phlebotomy.

• #2 Polycythaemia (Rubra) Vera – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/types-of-blood-cancer/myeloproliferative-neoplasms/polycythaemia-rubra-vera/

  Many people have no symptoms when they are first diagnosed with polycythaemia vera. […] If symptoms do develop, they tend to do so over time. They are mainly due to the increased thickness (hyperviscosity) and abnormally high numbers of blood cells in the circulating blood. Common symptoms include headaches, blurred vision, fatigue, weakness, dizziness, itchy skin and night sweats. […] Enlargement of the spleen (splenomegaly) is also common and occurs in around 75 per cent of cases. Symptoms include feelings of discomfort, pain or fullness in the upper left-side of the abdomen. […] Some people experience gout, which usually presents as a painful inflammation of the big toe or foot. […] In many cases, people with polycythaemia vera have a ruddy (red) complexion, and a reddening of the palms of the hand and soles of the feet, ear lobes, mucous membranes and the eyes.

• #2 Polycythemia Vera : Symptoms, Causes, Stages, Diagnosis and Treatment

  https://oncodaily.com/oncolibrary/cancer-types/225800

  Polycythemia vera often presents with non-specific symptoms such as fatigue, headaches, dizziness, and itching (pruritus). Fatigue is the most reported symptom, affecting daily activities and overall quality of life. Headaches and dizziness result from increased blood viscosity, which impairs proper blood flow to the brain. Itching, often worsened by warm showers or baths, is linked to elevated histamine levels due to abnormal blood cell production. […] One of the most serious risks of PV is the development of blood clots (thrombosis), which can occur in major veins or arteries. Increased blood viscosity heightens the risk of clots, potentially leading to life-threatening events such as strokes, heart attacks, or pulmonary embolisms. Bleeding episodes, though less common, can also occur due to platelet dysfunction despite elevated counts.

• #2 Erythrocytosis | eClinpath

  https://eclinpath.com/hematology/polycythemia/

  Erythrocytosis is defined as an increase in red blood cell (RBC) mass, usually absolute, and is also associated with an increased hematocrit (HCT) and hemoglobin concentration. […] An increased HCT or RBC count can be relative (proportional changes of RBC numbers in relation to plasma water) or absolute (a true increase in RBC numbers due to erythropoiesis, i.e. erythrocytosis). […] Absolute increase in HCT or RBC mass (erythrocytosis): This is due to increased erythropoiesis and can be primary (a bone marrow disorder) or secondary, due to appropriate or inappropriate production of the erythropoietic cytokine, erythropoietin. […] Secondary erythrocytosis: This is usually due to increased erythropoietin production. The physiologic stimulus for erythropoietin production is hypoxia, therefore erythropoietin production can be appropriate (in response to hypoxia), albeit excessive, or inappropriate (independent of hypoxia).

• #2 Secondary polycythemia: Its Symptoms, Diagnosis, Treatment, and Management

  https://www.webmd.com/a-to-z-guides/what-is-secondary-polycythemia

  Secondary polycythemia, also known as secondary erythrocytosis or secondary erythrocythemia, is a rare condition in which your body produces an excess amount of red blood cells. […] This thickened blood cant pass through your small blood vessels like capillaries easily. This increases your risk of having a stroke. […] Patients with secondary polycythemia may have symptoms including: Fatigue, Headache, Dizziness, Blurred vision, Anorexia, Weakness, Reduced mental acuity. […] Secondary polycythemia symptoms occur because of the thickening of your blood after red blood cells are overproduced; in some cases, the overproduction is accompanied by a decrease in the volume of plasma. […] Depending on the underlying cause (what caused secondary polycythemia), your symptoms may vary.

• #2 Erythrocytosis: Definition, Causes, Symptoms, and More

  https://resources.healthgrades.com/right-care/blood-conditions/erythrocytosis

  Erythrocytosis causes an increased number of red blood cells. Symptoms include weakness and tiredness. Treatment is vital to reduce the risks of heart attack, blood clots, and stroke. […] Many people with erythrocytosis feel weak and tired because the condition causes oxygen deprivation. […] Other common symptoms of erythrocytosis include headache, light-headedness, shortness of breath, and visual disturbances. […] Most cases of erythrocytosis are not serious, but some cases, such as polycythemia vera, require lifelong treatment to manage symptoms. Treatment for this condition is vital. Without treatment, you are more at risk for heart attacks, stroke, and blood clots. It is also highly important to address any underlying causes in secondary erythrocytosis. […] Erythrocytosis is a blood condition that causes an increased number of red blood cells. This condition can be primary or secondary.

• #2 Secondary Polycythemia: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/205039-overview

  In secondary polycythemia, the number of red blood cells (RBCs) is increased as a result of an underlying condition. […] Secondary polycythemia most often develops as a response to chronic hypoxemia, which triggers increased production of erythropoietin by the kidneys. […] To the extent that the increased RBCs alleviate tissue hypoxia, secondary polycythemia may in fact be beneficial. However, treatment with phlebotomy is indicated for patients with hematocrits higher than 60%-65%, who may experience symptoms such as impaired alertness, dizziness, headaches, and compromised exercise tolerance, and who may face increased risk for thrombosis, strokes, myocardial infarction, and deep venous thrombosis. […] At extreme levels of secondary polycythemia, patients can be at risk for thrombosis. Excessive polycythemia, usually defined as hematocrit levels higher than 65-70%, may result in increased whole blood viscosity. This, in turn, may lead to impaired blood flow locally, resulting in thrombosis. […] Hyperviscosity may also lead to generalized sluggish blood flow, resulting in impaired tissue oxygenation in multiple organs, which may lead to decreased mentation, fatigue, generalized weakness, and poor exercise tolerance.

• #2 Polycythemia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK526081/

  Polycythemia, also called erythrocytosis, refers to increased red blood cell mass, noted on laboratory evaluation as increased hemoglobin and hematocrit levels. […] The clinical significance of erythrocytosis, due to any cause, is related to the associated risk of thrombotic events due to hyperviscosity of blood. Additionally, in cases of polycythemia vera, there is potential for progression to leukemia. […] The clinical significance of erythrocytosis, due to any cause, lies in the associated risk of thrombotic events due to hyperviscosity of blood. Additionally, the potential for progression to leukemia in cases of polycythemia vera also warrants additional management strategies to be implemented. […] Progression to leukemia, particularly acute myeloid leukemia (AML), is seen in approximately 5% of cases and is often refractory to treatment. Studies have implicated the use of chlorambucil, pipobroman, or radioactive phosphorous as factors that increase the likelihood of progression.

• #2 Polycythaemia vera (PV) symptoms | Blood Cancer UK

  https://bloodcancer.org.uk/understanding-blood-cancer/polycythaemia-vera-pv/pv-symptoms/

  Having blood thats too thick can cause blood to clot too easily. This is known as thrombosis. This can be serious, so its important to know what to look out for. The symptoms of thrombosis will vary depending on where the clot is, but they include: slurred speech, weakness on one side of the body, drooping on one side of your face, sudden chest pain, dizziness and feeling short of breath, one leg or arm becoming painful, swollen and warm, coughing up blood, sudden blurred vision or loss of sight, sudden pain in the stomach area, possibly with swelling and signs of jaundice (a yellowing of the eyes, and yellowish skin in people with lighter skin tones).

• #2

  https://111.wales.nhs.uk/encyclopaedia/e/article/erythrocytosis

  Erythrocytosis also increases your risk of heart attack and stroke. Seek emergency medical help if you think that you or someone you’re with is having a heart attack or stroke. […] The outlook for erythrocytosis largely depends on the underlying cause. […] Many cases are mild and may not lead to any further complications. However, some cases particularly cases of polycythaemia vera can be more serious and require long-term treatment. […] If well controlled, erythrocytosis should not affect your life expectancy, and you should be able to live a normal life. However, people with polycythaemia vera can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes. […] Polycythaemia vera can also sometimes cause scarring of the bone marrow (myelofibrosis), which can eventually lead to you having too few blood cells. In some rare cases, the condition can develop into a type of cancer called acute myeloid leukaemia (AML).

• #2 Polycythemia: Definition, causes, symptoms, and more

  https://www.medicalnewstoday.com/articles/polycythemia

  Polycythemia can be managed with treatment. However, certain complications can turn into a life-threatening condition. […] In about 5% of cases, polycythemia may progress to difficult-to-treat leukemia. […] That said, having complications can affect a persons outlook. Some risk factors that can worsen the outlook include a history of blood clots, higher white blood cell counts, or the presence of immature forms of red and white cells in the blood. The latter two factors may indicate cancer.

• #2 Polycythaemia Rubra Vera (High Red Blood Cell Count)

  https://patient.info/allergies-blood-immune/polycythaemia-rubra-vera-high-red-blood-cell-count

  There is an increased risk of developing a blood clot (thrombosis) if you have PRV. […] Some people with PRV can develop bleeding problems, including nosebleeds, bleeding gums, bruising or bleeding from their gut. […] People with PRV may have itching of their skin (known as pruritus), particularly after having a bath or a shower. […] There is a chance that, after about 15 years, PRV can develop into a condition known as myelofibrosis. […] Therefore, if you have myelofibrosis, the number of red and white blood cells and platelets in your blood becomes too low. […] Between 6 and 15 in 100 people with PRV will develop myelofibrosis. Blood transfusions may be needed to keep up your red blood cell levels.

• #2 High Red Blood Cell Count (Polycythemia): Symptoms & Treatment

  https://www.emedicinehealth.com/polycythemia_high_red_blood_cell_count/article_em.htm

  Symptoms of polycythemia vera can be vague and quite general such as headache, easy bleeding, and more. […] Symptoms of polycythemia vera can be vague and quite general. Some of the most significant symptoms include: Easy bruising, Easy bleeding, Blood clot formation (potentially leading to heart attacks, strokes, and blood clots in the lungs [pulmonary embolism]), Bone and joint pain (hip pain or rib pain), Headache, Itching, Itching after taking a shower or bath (post-bath pruritus), Fatigue, Dizziness, Abdominal pain. […] People with primary polycythemia need to be aware of some of the potentially serious complications that may occur. The formation of blood clots (heart attacks, strokes, blood clots in the lungs [pulmonary embolism] or legs [deep vein thrombosis]) and uncontrolled hemorrhaging (nosebleeds, gastrointestinal bleeding) typically require prompt medical attention by the treating physician or emergency department. […] The outlook for primary polycythemia without treatment is generally poor, with a life expectancy of about 2 years. However, with phlebotomy alone, many patients lead normal lives and enjoy a normal life expectancy.

• #2 Erythrocytosis: What Is It, What Causes It, and More

  https://www.webmd.com/a-to-z-guides/what-is-erythrocytosis

  Many of the symptoms of erythrocytosis are caused by your blood flowing more slowly than normal. These symptoms can include: […] Blurred vision […] Headaches […] Confusion […] High blood pressure […] Nosebleeds […] Itching […] Weakness […] Tiredness.

• #2 Polycythemia: Definition, causes, symptoms, and more

  https://www.medicalnewstoday.com/articles/polycythemia

  It is not uncommon for a person to have polycythemia and be unaware of it. Symptoms tend to build very slowly over time. […] Having too many red blood cells thickens the blood, which makes healthy blood flow more difficult. It can also increase a persons risk of blood clots. This risk is especially high if a person with PV has an excess of both red blood cells and platelets. […] The symptoms of PV may become more apparent over time. More common symptoms include: dizziness or vertigo, headaches, excessive sweating, itchy skin, ringing in the ears, blurred vision, fatigue, reddish or purplish skin on the palms, earlobes, and nose, bleeding or bruising, a burning sensation in the feet, abdominal fullness, frequent nosebleeds, bleeding gums. […] Without treatment, people with PV may also be more likely to experience complications, such as: enlarged spleen, blood clots, angina, stroke, peptic ulcers, heart disease, gout, other blood disorders, such as myelofibrosis or leukemia.

• #2 Polycythemia Vera – Blood Disorders – MSD Manual Consumer Version

  https://www.msdmanuals.com/home/blood-disorders/myeloproliferative-disorders/polycythemia-vera

  People may feel tired and weak, light-headed, or short of breath, or develop symptoms caused by blood clots. […] Often, people with polycythemia vera have no symptoms for years. The earliest symptoms usually are weakness, tiredness, headache, light-headedness, shortness of breath, night sweats, itching after a shower or bath. […] A blood clot sometimes causes the first symptoms. The increase in red blood cells in polycythemia vera makes the blood thicker and more likely to clot more than normal. […] The excess of red blood cells may be associated with stomach ulcers, gout, and kidney stones. Rarely, polycythemia vera progresses to leukemia.

• #2 Polycythaemia vera | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/polycythaemia-vera

  Polycythaemia vera is characterised by the production of too many red blood cells. […] Typically, the onset of disease is sometime after the age of 50 years, with a slow but persistent progression. […] The symptoms of polycythaemia vera include: Headache, Dizzy spells, Itching skin, especially after bathing, Flushing of the skin, especially the face, Breathlessness, Phlebitis (inflammation of the vein), Vision problems, Skin rashes, Blue tinge to the skin (cyanosis), Fatigue, Gout, Kidney stones, Enlarged spleen (splenomegaly). […] Without treatment, around half of all people with symptomatic polycythaemia vera will die in less than two years. There is no cure, but treatment can extend the person’s life span by thinning the blood and reducing the risk of blood clots and other complications.

• #2 Polycythaemia vera – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/178

  Polycythaemia vera (PV) is generally a disease of middle and older age. […] Carries increased risks of thrombosis, haemorrhage, progression to myelofibrosis, and transformation to acute leukaemia. […] PV may progress to spent phase (post-PV myelofibrosis) and, uncommonly, to a devastating acute leukaemia. […] Life expectancy is reported to be modestly reduced compared with that of the general population. Leading causes of death are cardiovascular complications (including thrombosis and haemorrhage) and transformation to acute myeloid leukaemia. […] Other diagnostic factors include headache, generalised weakness/fatigue, pruritus, night sweats and bone pain, erythromelalgia, splenomegaly, plethora/ruddy cyanosis, tinnitus, blurry vision, arthralgia, abdominal discomfort, and hyperhidrosis (excessive sweating).

• #2 Secondary Polycythemia – MD Searchlight

  https://mdsearchlight.com/blood-disorders/secondary-polycythemia/

  Secondary polycythemia, a condition where your body produces too many red blood cells, often hints its presence through various signs and specific information in a patients history. Understanding the different causes of this condition can help doctors in their diagnosis. […] People with secondary polycythemia typically report symptoms like fatigue, headaches, and dizziness. Sometimes, the increased thickness of their blood may cause brief visual disturbances. […] During a physical examination, doctors might notice visible scratch marks due to itching, blue or purple tinted skin, and unusual nail and tooth conditions especially in smokers. […] In a research study by Galeas and colleagues, it was found that the average time from when a diagnosis was made to when a patient passed away was about 21.1 months. This was roughly half the time compared to patients with a disease known as polycythemia vera. The same study also found that about 5% of patients with a condition called secondary erythrocytosis experienced thrombotic episodes, which are blood clot incidents. However, the future health outcome or prognosis for patients was closely tied to the original cause of their illness and the occurrence of any complications.

• #2 Polycythemia Vera : Symptoms, Causes, Stages, Diagnosis and Treatment

  https://oncodaily.com/oncolibrary/cancer-types/225800

  Early symptoms of polycythemia vera are often subtle and non-specific, making timely diagnosis challenging. Weakness, shortness of breath, and blurred vision are common early indicators of PV. Weakness results from impaired oxygen delivery despite the increased red blood cell count, while shortness of breath may occur during physical exertion due to heightened blood viscosity. Blurred vision is often caused by reduced blood flow to the eyes. […] These vague symptoms are frequently misattributed to less serious conditions, leading to delays in diagnosis. […] In the proliferative phase, there is a marked increase in red blood cell production, often accompanied by elevated white blood cells and platelets. Symptoms such as fatigue, headaches, dizziness, itching, and an enlarged spleen (splenomegaly) are common. Thrombotic events, including strokes and deep vein thrombosis, become more likely due to increased blood viscosity.

• #2 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  Fatigue, insomnia, and difficulty concentrating are the most common presenting symptoms in patients with PV. […] Poor prognostic features include age older than 60 years, history of thrombosis, leukocytosis, high JAK2 burden, abnormal karyotype, and established cardiovascular risk factors such as smoking, hypertension, diabetes, obesity, and hyperlipidemia. […] Without treatment, death typically occurs within two years, mostly from thrombotic events. […] Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. […] For those diagnosed before 60 years of age, median survival is 24 years. […] The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. […] Progression to myelofibrosis over 15 years is 6% to 14%.

• #2 Erythrocytosis: Too Many Red Blood Cells

  https://www.verywellhealth.com/erythrocytosis-401324

  The prognosis for someone diagnosed with erythrocytosis is not dire, but a shorter life expectancy is common. A 2024 update on PV finds that people live an average of 15 years after their diagnosis, although that rises dramatically to 35 years or more if your diagnosis occurs before age 40. […] When too many red blood cells alter the mix of blood components, it can put you at risk for clots and other complications that can prove life-threatening. Conditions like PV and its complications may increase your symptoms and the need for treatment.

• #2 Erythrocytosis

  https://www.nhs.uk/conditions/erythrocytosis/

  Treatment for erythrocytosis aims to prevent symptoms and complications (such as blood clots), and treat any underlying causes. […] Some people may also need treatment for any other symptoms or complications of erythrocytosis they have, or for any underlying cause of the condition. […] The outlook for erythrocytosis largely depends on the underlying cause. […] Many cases are mild and may not lead to any further complications. However, some cases particularly cases of polycythaemia vera can be more serious and require long-term treatment. […] If well controlled, erythrocytosis should not affect your life expectancy, and you should be able to live a normal life. However, people with polycythaemia vera can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes.

• #2 Reddit – The heart of the internet

  https://www.reddit.com/r/polycythemiavera/comments/11evflu/primarysecondary_polycythemia_symptoms_and/

  In general, my HCT levels have been between 48-52 and my HGB levels have been between 16 -19.5 in the past two years. […] headaches […] HGB: Migraines appear at 16.5 +, debilitating migraines at 18.0+ […] blurred vision […] HGB: 19.4+ […] red skin particularly in the face, hands, and feet […] HGB: 16.5 ish+ […] tiredness […] HGB: feeling tired at 16.0 ish+, fatigued at 16.5+, exhausted and need bed rest at 18.0+ […] high blood pressure […] HGB: 18.0 […] dizziness […] HGB: mild dizziness at 16.5 ish+, high-level dizzy at 18.0+ […] confusion […] HGB: low levels at 16.5 ish+, high levels at 18.0+ish […] itchy skin especially after a bath or shower […] HGB: 16.0 ish+ […] tinnitus […] HGB: 16.0ish+ […] While all of the above symptoms can come and go independently of each other, sometimes many at one time, this and fatigue have been the only consistent symptoms that begin before all other symptoms and disappear as soon as I receive phlebotomy.

• #2 What is Erythrocytosis? – HealthTree for Myelofibrosis

  https://healthtree.org/blood-cancer/community/articles/what-is-erythrocytosis

  Erythrocytosis occurs when the red blood cells are extremely elevated, damaging normal blood circulation and the bodys function. […] Erythrocytosis is associated with hemoglobin levels over 16 g/dL, while when hemoglobin is less than 12 g/dL, it may indicate the presence of a condition called anemia. […] Erythrocytosis most often develops as a response to chronic low oxygen levels in the blood. The bodys response is to increase red blood cells to transport more oxygen. […] Large amounts of red blood cells can make the blood thicker and harder to flow through small blood vessels. […] Especially when hematocrit is higher than 60%-65%. These patients may experience symptoms such as impaired alertness, dizziness, headaches, and compromised exercise tolerance and may face increased risk for blood clots, strokes, heart infarction, and deep venous thrombosis. […] By removing some blood, phlebotomy helps: Reduce the number of red blood cells: This lowers your blood’s thickness and improves circulation. […] Decrease the risk of blood clots: Thick blood is more likely to form clots, which can be dangerous.

• #2 Diagnosing or Ruling Out Polycythemia Vera in Patients With Erythrocytosis – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/january-2019/diagnosing-or-ruling-out-polycythemia-vera-in-patients-with-erythrocytosis/

  Blood clots are the major complication in patients with polycythemia vera, and blood clots are even more common in those with Chuvash polycythemia. Common dogma dictates that a high hematocrit is the cause of blood thickening and blood clots, but I am skeptical that this is the major cause. No evidence exists that a high hematocrit is harmful and a direct cause of thrombosis; in addition, many conditions that lead to a very high hematocrit are not associated with thromboses.

• #2 Secondary Erythrocytosis – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/myeloproliferative-disorders/secondary-erythrocytosis

  Secondary erythrocytosis is erythrocytosis that develops secondary to disorders that cause tissue hypoxia, inappropriately increased erythropoietin production, or increased sensitivity to erythropoietin. […] Any elevation of hemoglobin or hematocrit above normal values for age and sex is considered erythrocytosis. […] Patients with chronic hypoxemia (arterial hemoglobin oxygen concentration 92%), typically due to lung disease, right-to-left intracardiac shunts, renal transplantation, prolonged exposure to high altitudes, or hypoventilation syndromes, often develop erythrocytosis. The primary treatment is to alleviate the underlying condition, but oxygen therapy may help, and phlebotomy may decrease viscosity and alleviate symptoms. […] In some people, phlebotomy is used to lower the number of red blood cells, but phlebotomy is rarely needed in secondary erythrocytosis.

• #2 Investigation and management of erythrocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7829024/

  Secondary erythrocytosis can be distinguished from PV in most patients with a focused clinical evaluation and, where available, determination of the erythropoietin level and JAK2 V617F mutation testing. Goals of treatment in PV are to alleviate symptoms, reduce the risk of thromboembolism, and monitor patients for transformation to myelofibrosis or acute leukemia. The majority of patients with PV should be treated with low-dose ASA and phlebotomy to achieve a target hematocrit value of less than 0.45. Cytoreduction, most commonly with hydroxyurea, should be considered in patients at high risk for thrombosis. Treatment of secondary erythrocytosis should be directed at the underlying cause.

• #2 Outcome of patient with Polycythemia Rubra Vera and psychiatric symptoms | Hematology, Transfusion and Cell Therapy

  https://www.htct.com.br/en-outcome-patient-with-polycythemia-rubra-articulo-S2531137920301036

  The treatment of mental symptoms in PV is refractory to psychiatric medication and responsive to hematological treatment, so that good hematological management implies control of mental status, avoids side effects of psychiatric medication and prevents thrombosis as a complication of PV. […] The case reports a patient with PV with the main occurrence of psychiatric symptoms. This presentation is described in the literature but isn’t considered usual for the disease. […] The blood count is important for the early diagnosis of the disease, due to its non-specific symptoms, representing the starting point for suspicion. […] Studies evaluating therapies in patients with PV and psychiatric symptoms show refractoriness to psychiatric medication and responsiveness to hematological treatment, as observed in the patient. In this case, there was a temporary remission of symptoms with the use of psychiatric medication, followed by relapse even with increased doses or association of new drugs. Only when cytoreduction started (with phlebotomies and hydroxyurea) was there complete remission, leaving the patient without the need for psychiatric medication.

• #2 Managing Symptoms of Polycythemia Vera – Living with and after blood cancer – Blood Cancer UK Online Community Forum

  https://forum.bloodcancer.org.uk/t/managing-symptoms-of-polycythemia-vera/2286

  I am experiencing a number of symptoms including fatigue, rashes/ itchiness, migraines, Reynolds disease, night sweats, a red face, elevated blood pressure, and infections. […] I have already started venesections so had anticipated some of the symptoms to abate. The only things to have improved is permanent fevers. […] I have PV and found that my migraines eased after a few venesections and my haematocrit fell within a normal range. […] Im always a little tired (I think thats because Im always a little iron deficient) and always a little itchy after a shower. […] I do suffer a lot with fatigue and itching but that seems common to most blood cancers. […] I feel very stressed and fatigued at the moment. Thirteen weeks of shielding are taking their toll.

• #2 Content – Health Encyclopedia – University of Rochester Medical Center

  https://www.urmc.rochester.edu/encyclopedia/content?ContentTypeID=90&ContentID=P02398

  Some children do not have symptoms, and polycythemia vera may be found when they’re having blood tests for other reasons. […] Symptoms can be different in each child. They can include: […] Headaches. […] Dizziness. […] Feeling weak and tired. […] Trouble breathing. […] Enlarged spleen. […] Trouble seeing. […] Itching or burning. […] Reddish color of the face. […] Bleeding gums. […] Losing weight. […] The symptoms of polycythemia vera can be like other health conditions. Make sure your child sees their health care provider for a diagnosis.

• #2 Hereditary Erythrocytosis Panel, Sequencing | Test Fact Sheet

  https://arupconsult.com/ati/hereditary-erythrocytosis-panel

  Erythrocytosis (ECYT) is characterized by an overproduction of red blood cells (RBCs), which leads to elevated hemoglobin and hematocrit levels. Symptoms may include headaches, dizziness, dyspnea, and epistaxis. The overabundance of RBCs may lead to hemorrhagic or thrombotic events, including myocardial infarction and deep vein thrombosis, although many individuals with ECYT experience mild symptoms and may be asymptomatic. […] Hereditary ECYT, also known as familial ECYT or congenital polycythemia, is a group of disorders in which ECYT is caused by inherited/germline pathogenic variants.

• #2 REVE2 – Overview: Erythrocytosis Evaluation, Blood

  https://www.mayocliniclabs.com/test-catalog/overview/618830

  The most common cause of hereditary erythrocytosis is the presence of a high-oxygen-affinity (HOA) hemoglobin variant. A subset of hemoglobins with increased oxygen (O2) affinity results in clinically evident erythrocytosis caused by decreased O2 unloading at the tissue level. Many are asymptomatic; however, some patients have recurrent headaches, dizziness, fatigue, and restless legs. A subset of patients experience thrombotic episodes. Affected individuals can be plethoric, and many are misclassified as polycythemia vera, particularly prior to more recent genetic testing availability. […] In addition, O2-sensing pathway variants, EPAS1(HIF2A) (OMIM 611783); EGLN1(PHD2) (OMIM 609820), and VHL (OMIM 263400) cause hereditary erythrocytosis and a subset are associated with pheochromocytoma and paragangliomas. All have shown an autosomal dominant pattern of inheritance, except VHL-associated erythrocytosis, which is an autosomal recessive disorder.

• #2 Outcome of patient with Polycythemia Rubra Vera and psychiatric symptoms | Hematology, Transfusion and Cell Therapy

  https://www.htct.com.br/en-outcome-patient-with-polycythemia-rubra-articulo-S2531137920301036

  Polycythemia Vera (PV) is a chronic myeloproliferative neoplasia generating an accumulation of erythrocytes in the peripheral blood (polyglobulia). The disease is described as polysymptomatic and its typical manifestations consist of headache, dizziness, weakness, plethora, itching and splenomegaly, the last three being more suggestive. Thus, a diagnosis of polycythemia may be suspect when it cannot be explained by a secondary cause. […] Neuropsychiatric symptoms are described in the literature but are less prevalent in current clinical practice. […] The pathophysiology of psychiatric events in PV is not well established, but the proposed models consist of two mechanisms resulting from blood hyperviscosity: (1) slowed blood flow with hypoxia and (2) multiple thrombosis, small and disseminated by the Central Nervous System (CNS).

• #3 Secondary Erythrocytosis – Blood Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/blood-disorders/myeloproliferative-disorders/secondary-erythrocytosis

  Erythrocytosis is increased production of red blood cells (erythrocytes). […] People with secondary erythrocytosis may have weakness, tiredness, headache, light-headedness, and shortness of breath.

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