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. […] Your provider may recommend you take a low dosage of aspirin regularly if youre at high risk of developing blood clots.

• #1 Screening and Prevention of Polycythemia Vera | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/screening-and-prevention-polycythemia-vera

  Primary polycythemia (polycythemia vera) can’t be prevented. However, with proper treatment, you can prevent or delay symptoms and complications. […] Sometimes you can prevent secondary polycythemia by avoiding things that deprive your body of oxygen for long periods. For example, you can avoid mountain climbing, living at a high altitude, or smoking. […] Following a healthy lifestyle to lower your risk of heart and lung diseases also will help you prevent secondary polycythemia.

• #1 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  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. […] Smoking cessation decreases the risk of thrombosis.

• #1 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 decisions are based on the patient’s risk for clotting complications (thrombosis). The two main risk factors for thrombosis are: A previous clot or clots, Age 60 years or older. […] Every patients medical situation is different and should be evaluated individually by a hematologist-oncologist. As you develop a treatment plan with your doctor, it is important to discuss: The results you can expect from treatment, Potential side effects, All treatment options, including treatments being studied in clinical trials.

• #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. […] 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. […] 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. […] 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.

• #1 Polycythemia Vera (PV): update on emerging treatment options | TCRM

  https://www.dovepress.com/polycythemia-vera-pv-update-on-emerging-treatment-options-peer-reviewed-fulltext-article-TCRM

  Current therapeutic first line recommendations based on risk adapted classification divided patients into two groups, according to age and presence of prior thrombotic events. […] Low-risk patients (age 60 years and no prior history of thrombosis) should be treated with aspirin (81100 mg/d) and phlebotomy, to maintain Hct 45%. […] High-risk patients (age 60 years and/or prior history of thrombosis), in addition to aspirin and phlebotomies, should receive cytoreductive therapy (HU) in order to reduce thrombotic risk. […] Nevertheless, it was also demonstrated that antiplatelet therapy, and not cytoreductive treatment, was significantly associated with lower risk of cardiovascular events. […] In refractory or first-line intolerant patients, ruxolitinib (RUX) can be used; treatments for advanced disease are scarce and chemotherapy, ie, busulfan, may be used as an option.

• #1 A guideline for the management of specific situations in polycythaemia vera and secondary erythrocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6519221/

  Thrombotic events are the major cause of morbidity and mortality in PV, and their prevention is the main objective of treatment. […] Both primary and secondary prevention should include control of cardiovascular risk factors in accordance with current recommendations (NICE, 2014). […] In accordance with evidence for the management of PV, low-dose aspirin should be offered to all patients (Landolfi et al, 2004). […] Indefinite anticoagulation for VTE is recommended because of the presence of continuing risk (NICE 2012; Watson et al, 2015; Kearon et al, 2016). […] For unprovoked VTE, where there is no physical precipitating factor, we therefore recommend indefinite anticoagulation to reduce the incidence of recurrence. […] Patients should be screened for hypertension, hyperlipidaemia, diabetes mellitus and a smoking history. (GRADE 1B) […] Cytoreduction and venesection to keep haematocrit (Hct) 045. (GRADE 1A) […] Consider low molecular weight heparin (LMWH) and/or interferon in addition to aspirin for high risk pregnancies (and for 6 weeks postpartum), based on individual patient discussion. (GRADE 1C)

• #1 Polycythemia Vera (PV): update on emerging treatment options | TCRM

  https://www.dovepress.com/polycythemia-vera-pv-update-on-emerging-treatment-options-peer-reviewed-fulltext-article-TCRM

  Polycythemia Vera (PV) treatment main goals are to control symptoms and prolong survival by preventing thrombosis, massive splenomegaly, and leukemic transformation. […] In order to reduce thrombotic risk, treatments should be addressed not only to normalize Hb, Hct and leukocytosis but also to decrease cardiovascular risk factors, such as obesity, diabetes, hypertension, dyslipidemia and smoking. Most importantly, hypertension and its management have a primary role: some studies, in fact, suggest that angiotensin converting enzyme inhibitors (ACE) inhibitors could also give a benefit in terms of reducing erythrocytosis. […] The cornerstone of thrombotic events prevention is represented by prophylaxis. In the multicenter ECLAP (European Collaboration on Low-Dose Aspirin in Polycythemia Vera) study, low dose aspirin (70100 mg per day) showed a significant reduction (60%) of combined risk of non-fatal myocardial infarction, non-fatal stroke, pulmonary embolism, major venous thrombosis and death from other cardiovascular causes.

• #1 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.

• #1 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.

• #1 Polycythemia Vera: What’s the Treatment?

  https://www.webmd.com/cancer/polycythemia-vera-treatments

  Daily aspirin use has some risks. It can make bleeding more likely, especially in the stomach and other parts of your digestive system. Talk to your doctor about these and other risks before you start to take aspirin regularly. […] This treatment slows red blood cell production in bone marrow, which thins your blood and helps it flow more easily. Yet doctors don’t use radiation therapy very often for PV because it also can make blood cancer (leukemia) more likely. […] Staying active will help keep your blood flowing and prevent clots. […] Drink a lot of water. Extra fluids will help prevent your blood from getting too thick.

• #1 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. […] Established PTE risk factors include male gender, normal hemoglobin/hematocrit pre-transplant (suggestive of robust native kidney erythropoietin production), renal artery stenosis, patients with a well-functioning graft, and dialysis before transplantation. […] 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. […] In this review article, we summarize the current literature in the field of post-transplant erythrocytosis after kidney transplantation.

• #1

  https://haematologica.org/article/view/haematol.2023.284658

  Hereditary or congenital erythrocytosis is a group of rare, inherited disorders, including congenital erythrocytosis associated with a germline mutation in erythropoietin receptor (EPOR). […] Our data from a homogeneous (both genetically and in terms of medical management) cohort of 33 affected subjects from the island of New Caledonia, a French territory in the South Pacific, suggest the possible benefit of phlebotomy and low-dose aspirin in the prevention of vascular events in patients with erythrocytosis due to an EPOR mutation. […] Among the 22 adults, 16 were being treated with a combination of aspirin and phlebotomy, two with a combination of anticoagulant therapy (for atrial fibrillation) and phlebotomy, one with phlebotomy alone, and three were not receiving any treatment. […] Overall, the use of aspirin (or oral anticoagulant in 2 patients) and phlebotomy was associated with a decreased risk of vascular events (P0.001, Fisher exact test).

• #1 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

  For people with polycythemia vera, treatment with the drug rusfertide appears to substantially reduce their reliance on blood draws to avoid serious health problems. […] Adding rusfertide to their ongoing treatment of phlebotomy had a major impact, Dr. Kremyanskaya said. […] For most participants who received rusfertide, [their] need for phlebotomy was significantly reduced or eliminated, she said, dropping from an average of nine phlebotomies per year before the trial to less than one phlebotomy per year after starting rusfertide. […] The goal of treatment is to maintain a hematocrit below 45%. […] People with polycythemia vera who are more likely to have problems like blood clots may receive additional treatments to reduce their red blood cell counts. […] 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.

• #1 Polycythemia Vera (PV): update on emerging treatment options | TCRM

  https://www.dovepress.com/polycythemia-vera-pv-update-on-emerging-treatment-options-peer-reviewed-fulltext-article-TCRM

  A recent systematic review and meta-analysis on RUX for prevention of thrombosis in PV were published by Masciulli et al showing an overall thrombosis annual incidence rate of 4.30% (95% CI, 3.005.60); the rate for BAT was 5.51% (95% CI, 3.727.30), and the rate for RUX was 3.09% (95% CI, 1.224.96). Authors concluded that the number of thrombotic events reported with RUX was consistently lower than that with BAT, but, globally, the difference did not reach significance. […] During the last years, ruxolitinib emerged as a new treatment in PV patients, as second line therapy: it appeared especially effective in patients with severe pruritus, symptomatic splenomegaly, or post-PV myelofibrosis symptoms. […] Many emerging treatments have been evaluated in the last years, especially for HU resistant patients. Unfortunately, conclusive results are still lacking. At present, givinostat seems to be the most promising novel drug and a phase III trial is ready to start in the near future.

• #1 Supplements for Polycythemia Vera: 5 To Take and 3 To Avoid | myMPNteam

  https://www.mympnteam.com/resources/supplements-for-polycythemia-vera-to-take-and-to-avoid

  If you have PV, your doctor may ask you to avoid some common vitamins and minerals because they can encourage the production of RBCs. Before you take any supplement, check with your doctor to make sure it doesn’t contain an ingredient you should avoid. […] You should never use supplements to replace your doctor-recommended PV treatment. Only FDA-approved medications and treatments for PV are evaluated for safety and effectiveness. Dietary supplements don’t have the same strict requirements as approved treatments. Additionally, there aren’t any clinical trials that show any supplement is safe or effective for PV. […] Before you start taking any new supplement, you should talk to your doctor about the possible benefits, side effects, and drug interactions with your current treatment.

• #1 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. […] Treatment for erythrocytosis depends on the cause and whether it is primary or secondary. […] Secondary erythrocytosis caused by oxygen deprivation is treated with oxygen therapy. People who smoke are advised to quit. […] In some cases of primary erythrocytosis, phlebotomy is used to reduce the number of red blood cells. […] Treatment for erythrocytosis is essential to lower your risk of heart attacks, blood clots, and stroke.

• #2 A guideline for the management of specific situations in polycythaemia vera and secondary erythrocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6519221/

  Thrombotic events are the major cause of morbidity and mortality in PV, and their prevention is the main objective of treatment. […] Both primary and secondary prevention should include control of cardiovascular risk factors in accordance with current recommendations (NICE, 2014). […] In accordance with evidence for the management of PV, low-dose aspirin should be offered to all patients (Landolfi et al, 2004). […] Indefinite anticoagulation for VTE is recommended because of the presence of continuing risk (NICE 2012; Watson et al, 2015; Kearon et al, 2016). […] For unprovoked VTE, where there is no physical precipitating factor, we therefore recommend indefinite anticoagulation to reduce the incidence of recurrence. […] Patients should be screened for hypertension, hyperlipidaemia, diabetes mellitus and a smoking history. (GRADE 1B) […] Cytoreduction and venesection to keep haematocrit (Hct) 045. (GRADE 1A) […] Consider low molecular weight heparin (LMWH) and/or interferon in addition to aspirin for high risk pregnancies (and for 6 weeks postpartum), based on individual patient discussion. (GRADE 1C)

• #2 Risk Factors for Disease Progression and Treatment Goals in Polycythemia Vera – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/january-2024-and-february-2024/risk-factors-for-disease-progression-and-treatment-goals-in-polycythemia-vera/

  Because thrombotic events contribute most to disease-related mortality, the primary goal of PV management is to minimize the risk of such events. Along with low-dose aspirin, which is recommended for all patients with PV, the cornerstone of management includes maintaining the HCT level below 45% using therapeutic phlebotomy; a variety of cytoreductive therapies including HU, pegrIFNα-2a or recombinant ropegylated interferon alpha 2b (ropegrIFNα-2b; Besremi, PharmaEssentia); or ruxolitinib. […] Accordingly, it is logical to hypothesize that normalizing HCT will improve this biological cascade and reduce the risk of thrombosis. […] The HCT target of less than 45%, which is included in the 2013 IWG-MRT response criteria, was established in 1978 by Pearson and colleagues and later reaffirmed by the CYTO-PV trial, which randomized patients to a strict (<45%) or liberal (45%-50%) HCT goal. [...] This confirmed the recommendation to maintain HCT below 45% with phlebotomy or the addition of cytoreduction in high-risk PV patients.

• #2 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.

• #2 Erythrocytosis treatment in Hyderabad | Dr. Karuna Kumar

  https://www.drkarunhematology.com/erythrocytosis.html

  There are two types of erythrocytosis: Primary erythrocytosis. This type is caused by a problem with cells in the bone marrow, where RBCs are produced. Primary erythrocytosis is sometimes inherited. […] Secondary erythrocytosis is an increase in RBCs caused by an underlying disease or the use of certain medications. […] 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.

• #2 Polycythemia Vera (PV): update on emerging treatment options | TCRM

  https://www.dovepress.com/polycythemia-vera-pv-update-on-emerging-treatment-options-peer-reviewed-fulltext-article-TCRM

  Polycythemia Vera (PV) treatment main goals are to control symptoms and prolong survival by preventing thrombosis, massive splenomegaly, and leukemic transformation. […] In order to reduce thrombotic risk, treatments should be addressed not only to normalize Hb, Hct and leukocytosis but also to decrease cardiovascular risk factors, such as obesity, diabetes, hypertension, dyslipidemia and smoking. Most importantly, hypertension and its management have a primary role: some studies, in fact, suggest that angiotensin converting enzyme inhibitors (ACE) inhibitors could also give a benefit in terms of reducing erythrocytosis. […] The cornerstone of thrombotic events prevention is represented by prophylaxis. In the multicenter ECLAP (European Collaboration on Low-Dose Aspirin in Polycythemia Vera) study, low dose aspirin (70100 mg per day) showed a significant reduction (60%) of combined risk of non-fatal myocardial infarction, non-fatal stroke, pulmonary embolism, major venous thrombosis and death from other cardiovascular causes.

• #2 Polycythemia Vera (PV) – Myeloid Neoplasms – Hematology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.15.6.

  The choice of treatment depends on the presence of risk factors for thrombotic complications (age 60 years and prior thrombosis). Phlebotomy is the primary therapy used in patients with marked erythrocytosis with the goal of Ht 45%. […] We recommend antiplatelet therapy (acetylsalicylic acid [ASA]) for all patients, unless oral anticoagulants are indicated. All patients should have cardiovascular risk factors addressed and modified. […] Antiplatelet therapy is recommended in all patients with no contraindications. […] Modification of cardiovascular risk factors: Prevention or treatment of hypertension, diabetes, obesity, and hypercholesterolemia; smoking cessation.

• #2 Screening and Prevention of Polycythemia Vera | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/screening-and-prevention-polycythemia-vera

  Primary polycythemia (polycythemia vera) can’t be prevented. However, with proper treatment, you can prevent or delay symptoms and complications. […] Sometimes you can prevent secondary polycythemia by avoiding things that deprive your body of oxygen for long periods. For example, you can avoid mountain climbing, living at a high altitude, or smoking. […] Following a healthy lifestyle to lower your risk of heart and lung diseases also will help you prevent secondary polycythemia.

• #2 Erythrocytosis – Causes, Symptoms, Diagnosis, and Treatment

  https://www.apollohospitals.com/diseases-and-conditions/erythrocytosis

  Preventing erythrocytosis involves addressing modifiable risk factors and maintaining overall health: […] Regular Check-Ups: Routine medical evaluations can help identify risk factors early. […] Healthy Lifestyle: Engaging in regular physical activity, maintaining a balanced diet, and avoiding smoking can reduce the risk of developing erythrocytosis. […] Recommendations: Hydration: Drink plenty of fluids, especially in hot weather or during physical activity. […] Avoid High Altitudes: If possible, limit exposure to high altitudes, especially for individuals with pre-existing conditions. […] Monitor Oxygen Levels: For individuals with chronic lung diseases, regular monitoring of oxygen levels can help manage erythrocytosis. […] Can erythrocytosis be prevented? While not all cases can be prevented, maintaining a healthy lifestyle, staying hydrated, and avoiding high altitudes can reduce the risk.

• #2 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.

• #2 TRT & Erythrocytosis – Facts and Recommendations – The Men’s Health Clinic

  https://themenshealthclinic.co.uk/trt-erythrocytosis-facts-recommendations/

  Erythrocytosis is the most commonly reported adverse event that occurs in testosterone trials, often a result of the TRT protocol. […] Testosterone can also act directly on the bone marrow and increase the number of EPO-responsive cells. […] Losing weight and cigarettes are the best thing you can do to prevent COPD and subsequent COPD induced polycythaemia. […] If you have polycythaemia then you can get therapeutic venesection (removing blood) to treat it. If you’re just generally trying to control HCT and have no known factors that would prevent you from donating blood, then donate blood; being on medically prescribed TRT will not prevent you from being able to donate blood.

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

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

  The widespread use of ACE-I and ARBs has been linked with the declining incidence of PTE. […] The foundation of PTE treatment is with either ACE-I or ARBs. […] As previously mentioned, the foundation of PTE treatment is with either ACE-I or ARBs. […] Given the potentially devastating complications of thromboembolic events associated with polycythemia, the KDIGO organization recommended treating PTE in their 2009 Transplant Recipient guidelines. […] While 20%-30% of patients may have long term resolution of their PTE, due to the large proportion of relapse, it is recommended that treatment, in most cases, continue indefinitely. […] 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.

• #2 Polycythemia Vera (PV): update on emerging treatment options | TCRM

  https://www.dovepress.com/polycythemia-vera-pv-update-on-emerging-treatment-options-peer-reviewed-fulltext-article-TCRM

  Current therapeutic first line recommendations based on risk adapted classification divided patients into two groups, according to age and presence of prior thrombotic events. […] Low-risk patients (age 60 years and no prior history of thrombosis) should be treated with aspirin (81100 mg/d) and phlebotomy, to maintain Hct 45%. […] High-risk patients (age 60 years and/or prior history of thrombosis), in addition to aspirin and phlebotomies, should receive cytoreductive therapy (HU) in order to reduce thrombotic risk. […] Nevertheless, it was also demonstrated that antiplatelet therapy, and not cytoreductive treatment, was significantly associated with lower risk of cardiovascular events. […] In refractory or first-line intolerant patients, ruxolitinib (RUX) can be used; treatments for advanced disease are scarce and chemotherapy, ie, busulfan, may be used as an option.

• #2 Polycythemia Vera in Pregnancy: A Descriptive Review of the Literature

  https://www.imrpress.com/journal/CEOG/50/4/10.31083/j.ceog5004077/htm

  Several organizations have published guidelines on the recommended management of PV during pregnancy, including the British Society for Haematology (BSH), European LeukemiaNet (ELN), National Comprehensive Cancer Network (NCCN), and Japanese Society of Hematology (JSH) […] Due to the concern for thrombosis in patients with PV, the main interventional strategies focus on prevention of thrombotic complications. Their guidelines direct that in otherwise low-risk pregnancies, it is recommended to keep the hematocrit within a gestational-appropriate range via serial phlebotomies, and to use low-dose aspirin throughout pregnancy and the postpartum period […] In pregnancies determined to be at higher risk for thrombosis, the recommendation is to initiate more intensive therapy by adding a prophylactic dose of low molecular weight heparin (LMWH)

• #2

  https://haematologica.org/article/view/haematol.2023.284658

  The occurrence of a myocardial infarction when phlebotomy was discontinued in one patient, and the cessation of miscarriages followed by two successful full-term pregnancies after the introduction of low-dose aspirin in another patient suggest the beneficial effect of these treatments in people with EPOR-related congenital erythrocytosis. […] Our results shed new light on the management of primary congenital erythrocytosis related to EPOR mutations and suggest that the combination of phlebotomy plus low-dose aspirin has a possible benefit in terms of preventing vascular events, thus providing an additional argument in line with recent recommendations.

• #2 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

  The findings from this trial are exciting, he said. But rusfertide is not yet approved by the Food and Drug Administration (FDA) to treat polycythemia vera. […] 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.

• #2 Erythrocytosis: Too Many Red Blood Cells

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

  Phlebotomy, a procedure to remove blood from a vein on a regular schedule, is often used to prevent complications with erythrocytosis. The blood is often replaced with normal saline. […] Other treatment options can include medications to prevent blood clots. Some options include: […] It’s important to note that the use of hydroxyurea, interferon, or ruxolitinib is only for patients with a PV diagnosis. These are not used in patients with erythrocytosis due to other causes. […] Certain lifestyle changes can help to lower cholesterol, improve blood pressure, and limit the risk of complications with erythrocytosis. For example, blood clots already are a risk to people with an erythrocytosis diagnosis, so limiting the additional factors that contribute to blood clots can help. […] Talk to your healthcare provider about smoking cessation and weight loss goals that can be managed with diet and exercise.

• #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. […] 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. […] 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 Polycythemia Vera: What’s the Treatment?

  https://www.webmd.com/cancer/polycythemia-vera-treatments

  Polycythemia vera (PV) causes your bone marrow to make too many red blood cells. You need these cells to carry oxygen around your body, but too many of them can make your blood thicken and form clots. Sometimes blood clots lead to a heart attack or stroke. […] Treatments lower the number of red blood cells and prevent blood clots. They also relieve other symptoms, like headaches and vision problems. With the right treatment, you can stay healthy — and feel better. […] Phlebotomy is the main PV treatment. It removes some of your blood to get rid of extra red blood cells. It also lowers your red blood cell count, so your blood thickness starts to get closer to normal. […] The goal is to lower your hematocrit level. Hematocrit is the percentage of red blood cells compared with the total amount of blood.

• #3 Polycythemia Vera (PV): update on emerging treatment options | TCRM

  https://www.dovepress.com/polycythemia-vera-pv-update-on-emerging-treatment-options-peer-reviewed-fulltext-article-TCRM

  Polycythemia Vera (PV) treatment main goals are to control symptoms and prolong survival by preventing thrombosis, massive splenomegaly, and leukemic transformation. […] In order to reduce thrombotic risk, treatments should be addressed not only to normalize Hb, Hct and leukocytosis but also to decrease cardiovascular risk factors, such as obesity, diabetes, hypertension, dyslipidemia and smoking. Most importantly, hypertension and its management have a primary role: some studies, in fact, suggest that angiotensin converting enzyme inhibitors (ACE) inhibitors could also give a benefit in terms of reducing erythrocytosis. […] The cornerstone of thrombotic events prevention is represented by prophylaxis. In the multicenter ECLAP (European Collaboration on Low-Dose Aspirin in Polycythemia Vera) study, low dose aspirin (70100 mg per day) showed a significant reduction (60%) of combined risk of non-fatal myocardial infarction, non-fatal stroke, pulmonary embolism, major venous thrombosis and death from other cardiovascular causes.

• #3 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.

• #3 Polycythemia vera – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/polycythemia-vera/

  Management of polycythemia vera focuses on preventing thrombotic and bleeding events and treating associated symptoms (e.g., pruritus, gout). […] Therapeutic phlebotomy and aspirin are indicated in all patients. Additionally, patients with high-risk polycythemia vera require cytoreductive therapies and, possibly, anticoagulation. […] The management of polycythemia vera focuses on decreasing the risk of thromboembolic events, which are the most common cause of mortality in patients with polycythemia vera. […] All patients: Start antiplatelet drugs (e.g., aspirin) unless contraindicated. […] High-risk polycythemia vera in patients with previous VTE: Start anticoagulants (e.g., parenteral anticoagulants, oral anticoagulants). […] Smoking cessation is associated with a decreased risk of thrombosis.

• #3 Risk Factors for Disease Progression and Treatment Goals in Polycythemia Vera – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/january-2024-and-february-2024/risk-factors-for-disease-progression-and-treatment-goals-in-polycythemia-vera/

  To achieve HCT control, phlebotomy is the current first-line therapy for patients with low-risk PV by ELN criteria. Administering cytoreductive agents in low-risk PV is recommended in certain patient populations, particularly those with symptomatic splenomegaly or high disease-related symptom burden, persistent leukocytosis, extreme thrombocytosis, or inadequate HCT control despite phlebotomy. […] A cytoreductive agent is recommended as a first-line treatment option in those with ELN high-risk PV unless contraindications exist. […] Low-dose aspirin is implemented into the treatment regimen in nearly all patients with PV unless there is a contraindication or the patient is receiving anticoagulation for a prior thrombotic event or comorbid condition. […] Nonetheless, implementation of low-dose aspirin is recommended in all patients with PV unless contraindicated, given the likely benefit, despite the weaknesses of prior studies.

• #3 Polycythemia Vera in Pregnancy: A Descriptive Review of the Literature

  https://www.imrpress.com/journal/CEOG/50/4/10.31083/j.ceog5004077/htm

  The use of interferon alpha (IFN-α) as a second-line agent to attempt cytoreductive therapy should also be a consideration in patients resistant to the above initial interventions […] A meta-analysis on the number of live births and maternal complications in pregnant patients with PV found that evidence of the benefit from aspirin or interferon treatment was of moderate quality […] They concluded that aggressive intervention with tight hematocrit control, aspirin, and LMWH is associated with significantly better outcomes for mother and infant […] It is therefore recommended to stop hydroxyurea at least 3 months before becoming pregnant. IFN-α is the treatment of choice in younger patients and pregnant patients due to its assumed safety in pregnancy and potential to achieve cytogenetic remission […] NCCN guidelines state that interferon alfa-2b, peginterferon alfa-2a, or peginterferon alfa-2b can be considered for higher risk pregnant patients requiring cytoreductive therapy.

• #3 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. […] In those with congenital defects, a number of serious thromboembolic events have been described, but there is little information available about outcomes in these individuals and, therefore, no evidence to guide management. 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 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). […] In congenital erythrocytoses, consideration needs to be given to venesection (to an achievable Hct level) and low-dose aspirin administration.

• #3 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. […] 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.

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