Materiały źródłowe

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

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

  Erythrocytosis is having a high concentration of red blood cells. Getting diagnosed and receiving treatment can prevent complications associated with erythrocytosis, like life-threatening blood clots. […] Its important to work with your provider to determine whats causing your erythrocytosis so you receive the right treatment to provide symptom relief or prevent complications, as needed. […] Most causes of erythrocytosis cant be cured. Instead, treatment can help ease symptoms. With more serious causes of erythrocytosis, your provider may provide treatments to prevent potential complications, like blood clots. […] Phlebotomy is the most common treatment for polycythemia vera. Your healthcare provider will remove blood to eliminate excess red blood cells and reduce your overall blood volume.

• #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. […] Venesection is the simplest and quickest way of reducing the number of red cells in your blood. It may be recommended if you have polycythaemia vera, a history of blood clots, or symptoms suggesting your blood is too thick. […] In cases of polycythaemia vera, medicine may be prescribed to slow down the production of red blood cells. […] If you have polycythaemia vera, daily low-dose aspirin tablets may be prescribed to help prevent blood clots and reduce the risk of serious complications. […] Some people may also need treatment for any other symptoms or complications of erythrocytosis they have, or for any underlying cause of the condition. […] As well as improving some cases of apparent erythrocytosis, making healthy lifestyle changes can also reduce the risk of potentially serious blood clots for people with all types of erythrocytosis.

• #3 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. […] To reduce the risk of thrombosis, most patients with PV are treated with low-dose acetylsalicylic acid and phlebotomy to achieve a target hematocrit value of less than 0.45, whereas patients at high risk for thrombosis may receive cytoreductive therapy. […] Treatment of secondary erythrocytosis should be directed at the underlying cause, and phlebotomy is not routinely recommended. […] The goals of treatment of PV are to reduce the risk of arterial and venous thromboembolism, and minimize symptoms. […] Patients with PV are treated with daily low-dose acetylsalicylic acid (ASA) and phlebotomy to achieve a target hematocrit value of less than 0.45 based on the results of 2 randomized trials.

• #4 Polycythemia Vera | AAFP

  https://www.aafp.org/pubs/afp/issues/2004/0501/p2139.html

  No single treatment is available for PV. Thrombosis accounts for the majority of morbidity and mortality. The major goal of treatment is to prevent thrombotic events. […] The mainstay of treatment for PV is phlebotomy, which is aimed at reducing hyperviscosity by decreasing the venous hematocrit level to less than 45 percent (0.45) in white men and 42 percent (0.42) in blacks and women. […] The use of myelosuppressive agents such as radioactive phosphorus (32P), chlorambucil (Leukeran), busulfan (Myleran), pipobroman (Vercyte), and hydroxyurea (Hydrea) in conjunction with phlebotomy has been studied. […] The nonalkylating myelosuppressive agent hydroxyurea is widely used in the treatment of PV, because it is less leukemogenic. […] Recombinant interferon alfa-2b reduces myeloproliferation and splenomegaly, and alleviates the symptom of pruritus.

• #5 Erythrocytosis: Definition, Causes, and Symptoms

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

  Treatment aims to reduce your risk of blood clots and relieve symptoms. It often involves lowering your RBC count. […] Treatments for erythrocytosis include: […] Phlebotomy (also called venesection). This procedure removes a small amount of blood from your body to lower the number of RBCs. You may need to have this treatment twice a week or more often until your condition is under control. […] Aspirin. Taking low doses of this everyday pain reliever may help prevent blood clots. […] Medications that lower RBC production. These include hydroxyurea (Hydrea), busulfan (Myleran), and interferon. […] Getting treatment that lowers the number of RBCs your body produces can reduce your symptoms and prevent complications.

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

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

  Your provider may recommend you take a low dosage of aspirin regularly if youre at high risk of developing blood clots. Your provider may also prescribe medicines that can lower your red blood cell count, including: Hydroxycarbamide, Hydroxyurea, Busulfan, Interferon alfa, Ruxolitinib. […] Your provider may recommend specific medications and procedures to treat the underlying condition causing erythrocytosis.

• #7 Diagnosis and Treatment of Erythrocytosis – touchONCOLOGY

  https://touchoncology.com/haematology/journal-articles/diagnosis-and-treatment-of-erythrocytosis/

  Venesection may be a therapeutic measure for those with congenital or idiopathic erythrocytosis. […] The only evidence-based target for Hct is 0.45, the advised target for those with PV on the basis of a retrospective study that showed that the incidence of thromboembolic events increased if Hct was above this cut-off. […] In PV the administration of low-dose aspirin has been shown to be beneficial, with a significantly reduced incidence of thromboembolic events in those on therapy in the European Collaboration on Low Dose Aspirin study (ECLAP).

• #8 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  First-line treatments, such as low-dose aspirin and goal-directed phlebotomy to a hematocrit level of less than 45% to reduce thrombotic events, improve quality of life and prolong survival. […] All patients should receive phlebotomy with a goal hematocrit level of less than 45%. […] All patients should receive daily low-dose aspirin (40 to 100 mg) in the absence of contraindications. […] Hydroxyurea is considered first-line cytoreductive therapy, if indicated. […] Hydroxyurea is the first-line agent for cytoreductive therapy in PV. […] A disease-oriented, uncontrolled study of 40 patients with high-risk PV reported that when pegylated interferon-alfa was used as first-line therapy, complete hematologic response (76% to 95%) and molecular response (18%) could be achieved over a 36-month period.

• #9 Polycythemia vera – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/polycythemia-vera/diagnosis-treatment/drc-20355855

  There’s no cure for polycythemia vera. Treatment aims to lower your risk of complications. Treatments also might ease your symptoms. […] The most common treatment for polycythemia vera is having blood withdrawn often. This is done using a needle in a vein, called phlebotomy. It’s the same procedure used for donating blood. […] If phlebotomy doesn’t help enough, these medicines can lower the number of red blood cells in your blood: Hydroxyurea (Droxia, Hydrea, Siklos), Interferon alfa-2b (Intron A), Ruxolitinib (Jakafi), Busulfan (Busulfex, Myleran). […] Your healthcare professional also will likely prescribe medicines to control risk factors for heart and blood vessel disease. These include high blood pressure, diabetes and high cholesterol.

• #10 Polycythemia Vera: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/205114-overview

  Management of polycythemia vera (PV) includes treatment measures such as phlebotomy to keep hematocrit below 45%, aspirin 81 mg daily, cytoreductive therapy for patients at high risk for thrombosis, and splenectomy in patients with painful splenomegaly or repeated episodes of splenic infarction. […] Hydroxyurea is the most commonly used cytoreductive agent. If hydroxyurea is not effective or not tolerated, alternatives include ropeginterferon alfa 2b, busulfan in patients older than 65 years, ruxolitinib (Jakafi), and fedratinib (Inrebic). […] The US Food and Drug Administration (FDA) has approved two Janus kinase (JAK) inhibitors for treatment of post-PV myelofibrosis. The JAK1 and JAK2 inhibitor ruxolitinib (Jakafi) was approved in 2011; the highly selective JAK2 inhibitor fedratinib (Inrebic) was approved in 2019.

• #11 Polycythemia Vera (PV) – MPN Research Foundation

  https://mpnresearchfoundation.org/polycythemia-vera-pv/

  Jakafi is the first FDA-approved treatment for PV patients who have an inadequate response to or cannot tolerate hydroxyurea. Jakafi inhibits the JAK 1 and 2 enzymes that are involved in regulating blood and immunological functioning. It also helps decrease the occurrence of an enlarged spleen (splenomegaly) and the need for phlebotomy. […] BESREMi was approved by the FDA in November 2021 as the first interferon specifically approved to treat adults with polycythemia vera regardless of their treatment history. […] Younger patients who require treatment and women of childbearing age are often treated with pegylated interferon because it has not been shown to cause birth defects.

• #12 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  A disease-oriented, retrospective study of 19 patients with PV and a median age of 76 years who had hydroxyurea resistance or intolerance and were started on busulfan as second-line therapy reported that 75% of patients had complete or partial hematologic response over a median of 3.8 years. […] A disease-oriented, randomized trial was conducted involving patients who were hydroxyurea resistant or intolerant. In these patients, ruxolitinib achieved better hematocrit control and spleen volume improvement compared with other single-drug alternatives selected by the investigator. […] Pruritus may be present in up to 68% of patients and is severe in up to 15% of patients. First-line therapy includes aspirin, antihistamines, and paroxetine (Paxil). […] All pregnant patients who have PV should receive low-dose aspirin, avoid iron supplementation in the absence of actual depletion, maintain gestational age-appropriate hematocrit levels, and be treated with enoxaparin (Lovenox) for six weeks postpartum if no contraindications occur.

• #13 Polycythemia Vera | AAFP

  https://www.aafp.org/pubs/afp/issues/2004/0501/p2139.html

  A risk-stratified approach to the management of PV is currently recommended. […] Patients treated with phlebotomy alone benefit from low rates of malignancy but experience more thrombosis events during the first few years of treatment. […] Therefore, stratifying patients by age and risk of thrombosis is useful. […] Consultation with a hematologist is recommended to apply such strategies, and newer agents may be tailored to patients on an individualized basis.

• #14 Investigation and management of erythrocytosis

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

  Observational studies suggest that patients with high-risk PV benefit from cytoreductive therapy in addition to low-dose ASA therapy and phlebotomy. […] Treatment should be directed at the underlying cause. There is no definitive evidence that the risk of thromboembolism is increased in patients with secondary erythrocytosis, and, therefore, phlebotomy is not recommended routinely.

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

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

  Secondary erythrocytosis may be treated with oxygen. […] People who smoke are advised to quit and are offered treatments to assist quitting. […] Any underlying disorder that is causing the oxygen deprivation and secondary erythrocytosis is treated as effectively as possible. […] In some people, phlebotomy (in which some of the person’s blood is removed) is used to lower the number of red blood cells. […] In tumor-associated erythrocytosis, removal of the tumor can be curative. […] Treating a specific hormone disorder or discontinuing a medication that can cause secondary erythrocytosis may also be curative. […] Relative erythrocytosis is treated by giving fluids by mouth or intravenously and by treating any underlying conditions that are contributing to the low plasma level.

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

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

  Treatment of secondary erythrocytosis is directed at the underlying disorder. For example, secondary erythrocytosis that is caused by oxygen deprivation may be treated with oxygen. People who smoke are advised to quit and are offered treatments to assist quitting. […] In some people, phlebotomy is used to lower the number of red blood cells, but phlebotomy is rarely needed in secondary erythrocytosis.

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

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

  Patients who have COPD are given low-flow oxygen therapy that can be used to correct the polycythemia-related hypoxia. […] Patients with chronic hypoxemia typically caused by lung disease, right-to-left intracardiac shunts, renal transplantation, prolonged exposure to high altitudes, or hypoventilation syndromes often develop secondary polycythemia. They can be treated with oxygen therapy and phlebotomy. […] Tumor-associated erythrocytosis, a type of secondary polycythemia, occurs because of tumors or cysts in the kidneys, tumors in the liver, cerebellar hemangioblastoma (a type of brain tumor), or uterine leiomyoma (a tumor in the womb). This condition can be treated by removing the lesion. […] Generally, secondary polycythemia on its own does not affect your lifespan, as long as the underlying condition is treated.

• #18 Secondary Polycythemia Treatment & Management: Medical Care

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

  Correction of the underlying cause of secondary polycythemia is the most important element of management. This may include cessation of exogenous erythropoietin, repair of arteriovenous or intracardiac shunts, or removal of tumors that are secreting erythropoietin. […] To restore viscosity and maintain circulation at its optimal level, phlebotomize or remove the offending red blood cells. Some patients with extreme secondary polycythemia have impaired alertness, dizziness, headaches, and compromised exercise tolerance. They may also be at increased risk for thrombosis, strokes, myocardial infarction, and deep venous thrombosis. These are the patients who require phlebotomy. […] Proper treatment of the underlying condition in polycythemia, when possible, is important, such as the following: Provide oxygen supplementation to patients with chronic obstructive pulmonary disease. Recommend weight loss in patients with obesity and hypoventilation. Recommend smoking cessation for patients with carboxyhemoglobin. Surgically correct arteriovenous shunts.

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

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

  Treatment for erythrocytosis depends on the cause. Primary erythrocytosis may be treated with phlebotomy, which is when some of your blood is taken out to remove excess red blood cells and improve your blood flow. This is the primary treatment for polycythemia vera. […] Secondary erythrocytosis is treated by treating the underlying cause. This treatment may include: Oxygen for people who have low oxygen levels, Advice and help to quit smoking, Treatment for any disorder that is causing low oxygen levels, Surgery to remove tumors, Stopping medicines that are causing the erythrocytosis, Treating a hormone disorder, Giving fluids.

• #20 Testosterone therapy-induced erythrocytosis: can phlebotomy be justified? in: Endocrine Connections Volume 13 Issue 10 (2024)

  https://ec.bioscientifica.com/view/journals/ec/13/10/EC-24-0283.xml

  Erythrocytosis, or elevated hematocrit, is a common side effect of testosterone therapy (TTh) in male hypogonadism. […] Several guidelines by endocrine organizations for the treatment of male hypogonadism recommend against starting TTh in patients presenting with elevated hematocrit at baseline or stopping TTh when its levels cannot be controlled. […] Besides dose adjustments, therapeutic phlebotomy or venesection is mentioned as a means of reducing hematocrit in these patients. […] However, evidence supporting the efficacy or safety of therapeutic phlebotomy in lowering hematocrit in TTh-induced erythrocytosis is lacking. […] The potential pros and cons should therefore be carefully weighed against each other, and shared decision-making is recommended for initiating therapeutic phlebotomy as a treatment in patients on TTh who present with increased hematocrit.

• #21 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20240221/Novel-treatment-for-polycythemia-vera-shows-promise-in-clinical-trial.aspx

  A novel treatment for polycythemia vera, a potentially fatal blood cancer, demonstrated the ability to control overproduction of red blood cells, the hallmark of this malignancy and many of its debilitating symptoms in a multi-center clinical trial led by the Icahn School of Medicine at Mount Sinai. […] In the phase 2 study, the drug rusfertide limited excess production of red blood cells, the main manifestation of polycythemia vera, over the 28-week course of treatment. The results suggest it could replace therapeutic phlebotomy, a common form of treatment which has proven to be a burden for many patients. […] Rusfertide appears to represent a significant step forward in treating polycythemia vera through its unique approach of limiting the amount of iron available for blood cell production.

• #22 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20240221/Novel-treatment-for-polycythemia-vera-shows-promise-in-clinical-trial.aspx

  Current therapies include aspirin; medications that can reduce red blood cells in the bloodstream, such as hydroxyurea, interferon, and ruxolitinib; and phlebotomy, which involves withdrawing blood using a needle in a vein to reduce blood volume. […] Frequent phlebotomies have proven onerous for patients due to the need for prolonged physician visits. […] Rusfertide was evaluated for safety and efficacy in 70 phlebotomy-dependent polycythemia vera patients. […] The researchers found that the agent was associated with improved and sustained control of hematocrit levels below 45 percent during the 28-week dose-discovery period, and was superior to placebo during the ensuing 12-week withdrawal period. […] Rusfertide shows great promise for achieving sustained hematocrit control in polycythemia vera patients. Just as importantly, it decreased the need for repeat phlebotomies, with some patients remaining virtually free of the procedure for more than two and a half years.

• #23 Rusfertide Cuts Number of Needed Blood Draws to Treat PCV – NCI

  https://www.cancer.gov/news-events/cancer-currents-blog/2024/rusfertide-polycythemia-vera-fewer-phlebotomies

  Treatment with rusfertide essentially tricks the body into behaving as if it has higher hepcidin levels, which causes iron levels to drop in the bloodstream, she continued. And without iron, red blood cell production stops. […] Indeed, findings from laboratory and animal model studies suggested that increasing hepcidin activity in the body could potentially limit red blood cell production. […] Before beginning treatment with rusfertide, the mean maximum hematocrit in participants was 50%. That level fell to 44.5% once rusfertide was added to their treatment. As a result, almost none of the participants needed a phlebotomy during the first part of the study. […] In addition, participants reported reduced severity of their symptoms, such as fatigue, night sweats, problems concentrating, and itching.

• #24 Rusfertide Cuts Number of Needed Blood Draws to Treat PCV – NCI

  https://www.cancer.gov/news-events/cancer-currents-blog/2024/rusfertide-polycythemia-vera-fewer-phlebotomies

  Rusfertide appears to help people with polycythemia vera maintain healthy hematocrit levels without the need for phlebotomy for more than 2 years, according to findings from the third and final part of the REVIVE trial, presented at the American Society of Hematology Annual Meeting in December 2023. […] Protagonist Therapeutics said it plans to seek FDA approval for rusfertide for treating polycythemia vera in late 2025. […] This is a new area of research in polycythemia vera that hopefully will lead to another [treatment option] for patients.

• #25

  https://journals.lww.com/md-journal/fulltext/2024/07190/the_efficacy_of_erythrocyte_apheresis_for.88.aspx

  To evaluate the efficacy of erythrocyte apheresis on the treatment of secondary erythrocytosis. […] Erythrocytic apheresis is effective and safe for the treatment of secondary erythrocytosis. […] In recent years, erythrocytapheresis has gradually replaced bloodletting for the treatment of erythrocytosis. […] Good therapeutic results have been achieved in clinical practice since the introduction of erythrocytapheresis. […] Compared with patients in the bloodletting group, headache, dizziness, and other discomfort feelings were relieved in the patients in the erythrocytapheresis group. […] The skin and mucous membrane cyanosis of the patients improved significantly after erythrocytapheresis. […] RBC and Hb levels decreased significantly more in the erythrocytapheresis group than in the bloodletting group after treatment.

• #26

  https://journals.lww.com/md-journal/fulltext/2024/07190/the_efficacy_of_erythrocyte_apheresis_for.88.aspx

  The follow-up results demonstrated that under hypoxic conditions, patients failed to improve significantly. […] Compared with traditional venous bloodletting therapy, erythrocyte apheresis is convenient, costs less, improves the efficiency of removing erythrocytes, reduces the number of times of bloodletting, shortens the treatment time, and significantly improves clinical symptoms of secondary erythrocytosis.

• #27 Diagnosis and management of congenital and idiopathic erythrocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3627324/

  Management of the specific disorder polycythemia vera has been discussed, and will change as newer treatments, such as JAK2 inhibitors, come into use. […] However, the management of a congenital or idiopathic erythrocytosis is much more problematic as there is little evidence available for guidance. Low-dose aspirin and venesection to an achievable target are the main therapeutic options that can be considered in the management of erythrocytosis. […] The therapeutic options for a patient with congenital and erythrocytosis are limited and largely of unproven efficacy. Low-dose aspirin in those without a specific contraindication and venesection to an achievable target HCT are the main treatments to be considered. Specific measures can be considered in situations such as high-affinity Hb. In the future JAK2 inhibitors may have a role in the treatment of Chuvash polycythemia.

• #28 Diagnosis and Treatment of Erythrocytosis – touchONCOLOGY

  https://touchoncology.com/haematology/journal-articles/diagnosis-and-treatment-of-erythrocytosis/

  An erythrocytosis arises when the red cell mass is increased. […] Investigation should commence with careful clinical evaluation. Determination of the erythropoietin level is then a first step to guide the further direction of investigation. […] In this group, consideration should be given to the use of venesection to attain an achievable haematocrit level, and also low-dose aspirin therapy. […] For patients with PV there are guidelines used in practice for their management. These include aspirin for all who can tolerate it, venesection to an Hct level of 0.45 and agents to reduce cell counts, directed by age, complications and symptoms. […] In congenital erythrocytoses, consideration needs to be given to venesection (to an achievable Hct level) and low-dose aspirin administration.

• #29

  https://link.springer.com/article/10.1007/s00277-021-04546-4

  Erythrocytosis has a diverse background. […] The aim of study was to retrospectively analyse the aetiology and management of non-clonal erythrocytosis patients referred to a haematology outpatient clinic in an 8-year period using a 3-step algorithm. […] Phlebotomies were performed in 56, 53 and 40% of patients in the SE, IE, and ICE group, respectively. […] Approximately 70% of patients in each group received aspirin. […] The treatment choice in selected patient groups is presented in Table 4. About 70% of patients in all three groups received aspirin. Phlebotomies were performed at least once in 56, 53 and 40% of patients in the SE, IE and ICE group, respectively. […] The treatment guidelines for non-clonal erythrocytosis are not clear. […] Treatment decisions in our study group were made on a case-by-case basis, after an individualized risk-benefit assessment and depending on the physicians belief.

• #30

  https://link.springer.com/article/10.1007/s00277-021-04546-4

  The main focus in non-clonal erythrocytosis, especially SE, should be identification of the cause and specific management, including discontinuation of a poor lifestyle habit such as smoking. […] According to British guidelines, patients with IE should be prescribed aspirin according to their cardiovascular risk factors, while phlebotomies can be used only in selected cases, but no target Hct is proven optimal. […] All in all, the type of congenital erythrocytosis, history of thromboembolic events, patients symptoms, and their alleviation after the procedure should be taken into consideration when deciding for phlebotomies.

• #31 https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/treatment

  https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/treatment

  Low-dose aspirin may reduce the risk of blood clots, heart attacks and strokes. Low-dose aspirin helps prevent platelets from sticking together, making it less likely for blood clots to form. […] Most PV patients have their blood drawn regularly to reduce the number of blood cells and decrease blood volume. Phlebotomy is a procedure in which blood is taken from a vein similarly to what is done when donating blood. […] High-risk PV patients may be prescribed cytoreductive drugs to reduce the number of blood cells. […] Treatment options include: Bathe less frequently, Bathe or shower in cool water and use a gentle soap, Avoid hot tubs, heated whirlpools and hot showers or baths, Keep skin well moisturized with lotion and try not to scratch it because that can damage the skin, Antihistamines such as diphenhydramine (Benadryl) or doxepin may help itching that does not go away, Light therapy (phototherapy) using a medicine called „psoralen” combined with ultraviolet A (UVA) light, Medications such as gabapentin or pregabalin that block neurotransmitters in the central nervous system from sending signals that trigger itching.

• #32 https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/treatment

  https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/treatment

  Polycythemia Vera (PV) is a chronic disease: It’s not curable, but it can usually be managed effectively for very long periods. The goal of therapy is to reduce the risk of thrombosis and to ease symptoms by lowering the number of extra blood cells. […] Many treatment options are designed to manage PV by lowering hematocrit levels below 45 percent for men and 42 percent for women. Careful medical supervision and therapy is important to keep the hematocrit concentration at normal levels. […] Treatment may include: Monitoring for new clots or bleeding, Manage cardiovascular risk factors, Low-dose aspirin, Phlebotomy. […] Treatment may include: Monitoring for new clots or bleeding, Manage cardiovascular risk factors, Low-dose aspirin, Phlebotomy, Medication to reduce the number of blood cells (cytoreductive medication).

• #33 JAK2 unmutated erythrocytosis: current diagnostic approach and therapeutic views | Leukemia

  https://www.nature.com/articles/s41375-021-01290-6

  In terms of management, optimal hypertension control with lisinopril was emphasized and low-dose aspirin initiated. […] The need for periodic phlebotomy was based upon symptoms. […] Phlebotomy should be reserved for relief of symptoms with documented response to the particular treatment modality; reported symptoms in patients with non-clonal erythrocytosis include fatigue, generalized weakness, headaches, visual changes, mental fog, tinnitus, chest pain, palpitations, dyspnea, abdominal and bone pain. […] Aspirin 81mg daily should be instituted in the presence of cardiovascular risk factors. […] The institution of phlebotomy in such cases requires careful assessment of risk-benefit balance. […] Phlebotomy with volume replacement should be used sparingly for mere symptom relief only when Hct rises above 65%.

• #34 Testosterone therapy and secondary erythrocytosis | International Journal of Impotence Research

  https://www.nature.com/articles/s41443-021-00509-5

  Secondary erythrocytosis is one of the most common adverse events associated with testosterone therapy (TT). Upon encountering this, clinicians will often either adjust TT dosing, stop therapy, order a phlebotomy, or recommend a combination of these. […] Despite this, the evidence for secondary polycythemia causing harm during TT is scarce, and the hematocrit-based cutoffs present in multiple guidelines appear to be arbitrarily chosen. […] we present the pathophysiology behind TT and secondary erythrocytosis, the evidence connecting TT, secondary erythrocytosis and major adverse cardiovascular events (MACE), and the data supporting varying interventions upon diagnosis of secondary erythrocytosis.

• #35 Testosterone therapy-induced erythrocytosis: can phlebotomy be justified? in: Endocrine Connections Volume 13 Issue 10 (2024)

  https://ec.bioscientifica.com/view/journals/ec/13/10/EC-24-0283.xml

  Several endocrine organizations mention therapeutic phlebotomy as a means of treating TTh-induced erythrocytosis, commonly with a hematocrit cutoff around 54%, as an alternative or adjuvant to testosterone dosage reduction. […] Periodic phlebotomy to persistently decrease hematocrit (even within the physiological range) has two consequences that we will discuss: a decrease in tissue pO2 and depletion of iron stores. […] In summary, the practice of therapeutic phlebotomy for treating TTh-induced erythrocytosis might potentially increase, rather than decrease, thrombotic risk through the HIF pathway by the combined action of depleting iron stores and decreasing tissue pO2.

• #36 Post-transplant erythrocytosis after kidney transplantation: A review

  https://www.wjgnet.com/2220-3230/full/v11/i6/220.htm

  Post-transplant erythrocytosis (PTE) is defined as persistently elevated hemoglobin 17 g/dL or hematocrit levels 51% following kidney transplantation, independent of duration. […] Angiotensin converting enzyme inhibitors (ACE-I) and angiotensin receptor blockers (ARBs) are the mainstays of treatment. Increased ACE-I/ARB use has likely contributed to the falling incidence of erythrocytosis. […] The foundation of PTE treatment is with either ACE-I or ARBs. RAAS inactivation has been documented to cause dose-dependent decrease in Hct levels, though the exact mechanism behind this decrease is not fully understood. […] In patients that either cannot tolerate ACE-I/ARBs, have contraindications to them, or do not have a treatment response, second line therapies include phlebotomy or theophylline.

• #37 Post-transplant erythrocytosis after kidney transplantation: A review

  https://www.wjgnet.com/2220-3230/full/v11/i6/220.htm

  As a final line of therapy for patients who prefer to avoid both phlebotomy and theophylline, patients can have their antiproliferative immunosuppressive agent switched from mycophenolate to sirolimus, as PTE was found to be less prevalent in patient who were administered the latter agent. […] In brief, several studies have shown that ACE-I/ARBs are first-line therapy, phlebotomy is second-line, and that theophylline is a limited alternative both in terms of efficacy and tolerance.

• #38 Polycythemia Vera Diagnosis & Treatment – The Patient Story

  https://thepatientstory.com/mpn/polycythemia-vera/diagnosis/

  Polycythemia vera is a complex disease to diagnose as it often does not present any symptoms. Many cases of PV are caught during a routine physical or blood work. After that, a multitude of tests are run to verify the diagnosis. […] This article looks at the tests doctors run to diagnose a patient with polycythemia vera. In addition, we go into a wide variety of treatment plans that doctors use to treat PV, along with some things you can do in your day-to-day life to help manage symptoms. […] While there is currently no cure for polycythemia vera, several treatment plans help reduce the risk of complications and ease symptoms. According to Cleveland Clinic and Mayo Clinic, treatments for polycythemia vera include: Blood withdrawals (phlebotomy), Aspirin, Treatments aimed at reducing chronic itching (pruritus), Drugs that reduce red blood cell count, Bone marrow transplant, Experimental treatments (clinical trials).

• #39 Extreme erythrocytosis in a cat reverts after short-term treatment with hydroxyurea and the cat remains healthy for years in: Journal of the American Veterinary Medical Association Volume 262 Issue 5 (2024)

  https://avmajournals.avma.org/view/journals/javma/262/5/javma.23.11.0659.xml

  Given the apparent response to treatment and lack of adverse effects to hydroxyurea, treatment was continued at a reduced interval (every other day). […] Two months after starting treatment, the Hct had normalized (41%), and the cat was doing well at home. […] Low-dose hydroxyurea therapy was discontinued after 2.5 years. And 3.5 years after initial diagnosis of erythrocytosis, and 10 months after discontinuation of hydroxyurea treatment, neither clinical signs nor erythrocytosis recurred. […] The authors acknowledge that while hydroxyurea treatment may have initially helped, it appears that an unidentified underlying secondary cause of erythrocytosis was initially present, but later resolved. […] Severe erythrocytosis, even if not caused by dehydration, may be reversible, and discontinuation of treatment may be possible in select cases.

• #40 Primary Erythrocytosis in a Dog | Today’s Veterinary Practice

  https://todaysveterinarypractice.com/hematology/primary-erythrocytosis-in-a-dog/

  The goal of treatment for erythrocytosis is to maintain a PCV of 55% or lower to eliminate or minimize signs of hyperviscosity. The cornerstone of treatment for absolute erythrocytosis in patients for which the underlying cause cannot be identified or resolved in both human and veterinary medicine is phlebotomy. The typical volume removed is 15 to 20 mL/kg. In some patients, intermittent phlebotomy is the only treatment necessary to achieve control of PCV and clinical signs. Placement of a vascular access port can facilitate phlebotomy in patients for which frequent hospital visits and restraint are not unduly stressful. […] In dogs, hydroxyurea is used to control RBC mass in patients with absolute erythrocytosis (primary or secondary) for which the cause of the erythrocytosis cannot be resolved and for which the required interval between phlebotomies is unacceptably short. For some patients, the disease may be controlled with hydroxyurea alone, whereas some may require concurrent intermittent phlebotomies to maintain a PCV within the target range. Various dosing regimens have been reported, including 40 to 50 mg/kg q48h with titration based on monitoring or a loading dose of 30 mg/kg q24h for 7 days followed by a maintenance dose of 15 mg/kg q24h. Hydroxyurea is usually well tolerated. Reported adverse effects in dogs include myelosuppression, gastrointestinal upset, dermatologic reactions, and onychomadesis (toenail loss).

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