Materiały źródłowe

• #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 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).

• #2 Polycythemia Vera: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17742-polycythemia-vera

  Polycythemia vera is a chronic condition with no cure, but medical care can help you manage symptoms and the risk of complications. […] In the early stages, PV is treated conservatively. Typical treatments include: […] The most common treatment for PV is to have regular blood withdrawals. Its the same procedure you would have to donate blood. A healthcare technician inserts a needle into a vein in your arm and withdraws a small amount of blood usually a pint, but it may vary depending on your condition. This reduces your overall blood volume and your number of excess blood cells. […] This over-the-counter medication is commonly prescribed to reduce the risk of blood clotting. A low daily dose of aspirin helps prevent blood platelets from sticking together. […] If your symptoms have become more severe, or if you have a history of thrombosis (blood clotting) and are considered higher risk, you may be offered additional treatment options.

• #3 How is Polycythemia Vera Treated? | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/how-polycythemia-vera-treated

  Polycythemia vera (PV) doesn’t have a cure. However, treatments can help control the disease and its complications. PV is treated with procedures, medicines, and other methods. You may need one or more treatments to manage the disease. […] The goals of treating PV are to control symptoms and reduce the risk of complications, especially heart attack and stroke. To do this, PV treatments reduce the number of red blood cells and the level of hemoglobin (an iron-rich protein) in the blood. This brings the thickness of your blood closer to normal. […] Phlebotomy reduces your red blood cell count and starts to bring your blood thickness closer to normal. […] Your doctor may prescribe medicines to keep your bone marrow from making too many red blood cells. Examples of these medicines include hydroxyurea and interferon-alpha.

• #4 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 the condition causes itching, medicines such as antihistamines or treatments with ultraviolet light might give you relief. […] 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.

• #5 Polycythemia – StatPearls – NCBI Bookshelf

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

  The treatment of secondary polycythemia is directed at correcting the cause. […] For polycythemia vera, available treatment modalities include: […] Phlebotomy was established as the backbone of therapy, primarily based on the trial conducted by the Polycythemia Vera Study Group (PVSG). The study found that, compared to chlorambucil or radioactive phosphorous treatment, treatment with phlebotomy alone was associated with longer median survival. […] The rationale behind repeated phlebotomies was that cytoreduction would reduce hyperviscosity. Additionally, it would induce a state of iron deficiency that would help retard red-cell proliferation. […] In practice, weekly sessions are conducted, during which approximately 500 mL of blood is removed, provided the hemodynamic status permits this.

• #6 Polycythemia Vera: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17742-polycythemia-vera

  Polycythemia vera is a chronic condition with no cure, but medical care can help you manage symptoms and the risk of complications. […] In the early stages, PV is treated conservatively. Typical treatments include: […] The most common treatment for PV is to have regular blood withdrawals. Its the same procedure you would have to donate blood. A healthcare technician inserts a needle into a vein in your arm and withdraws a small amount of blood usually a pint, but it may vary depending on your condition. This reduces your overall blood volume and your number of excess blood cells. […] This over-the-counter medication is commonly prescribed to reduce the risk of blood clotting. A low daily dose of aspirin helps prevent blood platelets from sticking together. […] If your symptoms have become more severe, or if you have a history of thrombosis (blood clotting) and are considered higher risk, you may be offered additional treatment options.

• #7 Polycythemia – StatPearls – NCBI Bookshelf

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

  This is continued weekly until a target hematocrit of under 45% is obtained. […] For secondary polycythemias, phlebotomy is usually reserved for the following conditions: Chronic lung diseases, Cyanotic heart diseases, Post-renal transplant patients with hypertension and erythrocytosis, not responding to optimal doses of angiotensin-converting enzyme inhibitors (ACEIs)/angiotensin receptor blockers (ARBs). […] Hydroxyurea is usually considered second-line therapy. Evidence of benefit came from, among others, a study by the Polycythemia Vera Study Group (PVSG) that showed lower rates of thrombosis compared to a historical cohort treated with phlebotomy alone. […] Indications for use include: Poor venous access, High phlebotomy requirement, When phlebotomy is not possible due to logistic reasons, Severe thrombocytosis, Intractable pruritus.

• #8 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).

• #9 Polycythaemia vera (PV) | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/blood-cancer/polycythaemia-vera-pv

  Treatment for polycythaemia vera (PV) is usually given to prevent problems such as blood clots. This is called supportive treatment. Treatment can also be given to control PV by reducing the number of red blood cells (cytoreductive therapy). You will have regular blood tests to check for changes in your condition. […] Before treatment, a team of health professionals work together to plan the treatment they feel is best for you. This team is called a multi-disciplinary team (MDT). […] Most people with PV are treated with aspirin tablets. Aspirin affects the way platelets stick together and helps prevent blood clots. Drugs that prevent clots are called anti-platelet drugs or blood thinners. Aspirin does not affect the number of platelets in the blood. […] Regularly removing about a pint (500ml) of blood is a common treatment for PV. It reduces the number of red blood cells in the blood and makes the blood thinner.

• #10 Polycythemia Vera: A Comprehensive Approach to Management – Hematology Advisor

  https://www.hematologyadvisor.com/features/polycythemia-vera-how-to-treat-approach-management-risk/

  In general, the first-line treatment of choice for cytoreductive therapy for PV is hydroxyurea in conjunction with therapeutic phlebotomy to maintain a goal hematocrit of 45% based on the CYTO-PV study, which demonstrated a reduced rate of cardiovascular events as compared to less-stringent hematocrit control (45%-50%). […] Pegylated interferon alfa is a reasonable first-line alternative to hydroxyurea and may be preferred in very young patients. […] In these special populations, pegylated interferon alfa-2a is a reasonable safer PV treatment option. […] Evidence-based treatment strategies are recommended to maximize life expectancy. […] The potential for combination therapy with ruxolitinib and pegylated interferon alfa-2a is also being explored. […] New treatment options are in development to address the current unmet needs in PV, including ropeginterferon and givinostat, both in phase 3 clinical development.

• #11 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).

• #12 Polycythemia Vera Treatment & Management: Approach Considerations, Medical Care, Phlebotomy

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

  Patients with thrombotic tendencies or those who develop thrombocytosis after phlebotomy should be treated with marrow suppression; consider anagrelide in younger patients (aged 50-70 y). […] Maintain blood values at reference range levels by regular examination and treatment. […] All patients with PV should undergo phlebotomy to keep their hematocrit below 45%. […] All patients with PV should take aspirin, 81 mg daily, unless contraindicated by major bleeding or gastric intolerance. […] If a patient is at high risk for thrombosis, cytoreductive therapy is added to the management plan. Hydroxyurea at a starting dose of 500 mg twice daily is the most commonly used cytoreductive agent. […] In patients whose PV is refractory to hydroxyurea or who cannot tolerate the drug, interferon alpha can be used as an alternative.

• #13 How is Polycythemia Vera Treated? | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/how-polycythemia-vera-treated

  Hydroxyurea is a medicine generally used to treat cancer. This medicine can reduce the number of red blood cells and platelets in your blood. As a result, this medicine helps improve your blood flow and bring the thickness of your blood closer to normal. […] Interferon-alpha can prompt your immune system to fight overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Radiation treatment can help suppress overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Aspirin can relieve bone pain and burning feelings in your hands or feet that you may have as a result of PV. Aspirin also thins your blood, so it reduces the risk of blood clots.

• #14 Polycythemia vera therapy | BLCTT

  https://www.dovepress.com/polycythemia-vera-barriers-to-and-strategies-for-optimal-management-peer-reviewed-fulltext-article-BLCTT

  Venesection (VS) remains the cornerstone treatment for PV. Removing red blood cells from the peripheral blood is primarily aimed at reducing blood viscosity, thus preventing TE with associated complications and helping to alleviate symptoms common to PV, such as pruritus, headache, and fatigue. […] Thus, beyond the risk stratification, VS is indicated in PV patients with elevated Hct levels (45%). In case of persistent symptomatic burden (erythromelalgia, amaurosis, persistent headache) or particular circumstances (pregnancy due to hemostatic changes, the greater thrombotic risk), the therapeutic target could also be lower (30-39%). […] The use of aspirin in PV has historically been debated. […] Direct oral anticoagulation (DOAC) therapy usage has been raised in the last year to prevent and treat TE in cardiovascular diseases.

• #15 Polycythemia vera: historical oversights, diagnostic details, and therapeutic views | Leukemia

  https://www.nature.com/articles/s41375-021-01401-3

  Additional treatment options include busulfan and ruxolitinib; the former is preferred in older patients and the latter in the presence of symptoms reminiscent of post-PV myelofibrosis or protracted pruritus. […] The cornerstone of treatment in PV includes scheduled phlebotomy, with a Hct target of 45%, and daily low-dose aspirin therapy, in all patients, regardless of risk category. […] The therapeutic role of aspirin in the treatment of PV has long being suspected and was initially faced with some concerns regarding bleeding complications, but its formal transition into routine clinical practice was facilitated by a controlled study from the ECLAP group. […] In general, cytoreductive therapy is not indicated for low-risk disease and its lack of additional value was indirectly surmised from a controlled study in ET. However, aggressive phlebotomy in low-risk patients with PV might result in severe phlebotomy-induced side effects and might also not be adequate in controlling certain disease-associated symptoms such as severe pruritus.

• #16 How is Polycythemia Vera Treated? | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/how-polycythemia-vera-treated

  Hydroxyurea is a medicine generally used to treat cancer. This medicine can reduce the number of red blood cells and platelets in your blood. As a result, this medicine helps improve your blood flow and bring the thickness of your blood closer to normal. […] Interferon-alpha can prompt your immune system to fight overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Radiation treatment can help suppress overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Aspirin can relieve bone pain and burning feelings in your hands or feet that you may have as a result of PV. Aspirin also thins your blood, so it reduces the risk of blood clots.

• #17 Polycythemia Vera Treatment & Management: Approach Considerations, Medical Care, Phlebotomy

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

  Patients with thrombotic tendencies or those who develop thrombocytosis after phlebotomy should be treated with marrow suppression; consider anagrelide in younger patients (aged 50-70 y). […] Maintain blood values at reference range levels by regular examination and treatment. […] All patients with PV should undergo phlebotomy to keep their hematocrit below 45%. […] All patients with PV should take aspirin, 81 mg daily, unless contraindicated by major bleeding or gastric intolerance. […] If a patient is at high risk for thrombosis, cytoreductive therapy is added to the management plan. Hydroxyurea at a starting dose of 500 mg twice daily is the most commonly used cytoreductive agent. […] In patients whose PV is refractory to hydroxyurea or who cannot tolerate the drug, interferon alpha can be used as an alternative.

• #18 Polycythaemia Vera: Causes, Symptoms, and Treatment

  https://patient.info/doctor/polycythaemia-vera-pro

  Intermittent long-term phlebotomy to maintain the haematocrit below 45% (lower target level may be appropriate for women). Phlebotomy may cause progressive and sometimes severe thrombocytosis and iron deficiency. Splenomegaly and pruritus may persist despite control of the haematocrit by phlebotomy. […] Low-dose aspirin produces a small reduction in thrombotic events, including myocardial infarction and stroke, whilst not increasing the risk of haemorrhage. […] If it is not possible to control thrombotic events with phlebotomy alone then myelosuppression must be considered. However, this is not without risk and increases the risk of leukaemic transformation. Risks and benefits have to be balanced. […] Cytoreductive therapy is recommended for people at high risk of thrombosis (aged over 60 years, or history of thrombosis).

• #19 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 the condition causes itching, medicines such as antihistamines or treatments with ultraviolet light might give you relief. […] 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.

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

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

  Management of polycythemia vera (PV) treatment measures are as follows: […] Phlebotomy to keep hematocrit below 45% […] Aspirin 81 mg daily […] Cytoreductive therapy for patients at high risk for thrombosis […] 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 the following: […] Ropeginterferon alfa 2b […] Busulfan in patients older than 65 years […] Ruxolitinib (Jakafi) […] Fedratinib (Inrebic).

• #21 How is Polycythemia Vera Treated? | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/how-polycythemia-vera-treated

  Hydroxyurea is a medicine generally used to treat cancer. This medicine can reduce the number of red blood cells and platelets in your blood. As a result, this medicine helps improve your blood flow and bring the thickness of your blood closer to normal. […] Interferon-alpha can prompt your immune system to fight overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Radiation treatment can help suppress overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Aspirin can relieve bone pain and burning feelings in your hands or feet that you may have as a result of PV. Aspirin also thins your blood, so it reduces the risk of blood clots.

• #22 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. 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. […] This 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. […] After your red blood cell level drops, you’ll take one of these prescription drugs to slow the production of new red blood cells in your bone marrow. Hydroxyurea is a cancer drug that slows down the growth of new cells in your body. In PV, it lowers the number of red blood cells and platelets, which are cells that help blood clot. If you take this drug, you may not need phlebotomy.

• #23 Polycythemia – StatPearls – NCBI Bookshelf

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

  The standard daily doses range from 500 to 1500 mg per day. […] The JAK2 inhibitor ruxolitinib is used when patients are intolerant or unresponsive to hydroxyurea. […] Evidence supporting the use of Ruxolitinib in myeloproliferative disorders came from the COMFORT trials. […] The standard recommended dose for polycythemia vera is 10 mg twice a day. […] Low-dose aspirin is indicated when there is inadequate control of microvascular symptoms after achieving the target hematocrit or in the presence of other cardiovascular risk factors. […] Depending on the severity of pruritus and the clinical response to therapy, therapeutic modalities available for symptomatic relief include antihistamines and selective serotonin reuptake inhibitors (SSRIs). […] The standard therapeutic measures of phlebotomy and low-dose aspirin are appropriate in most cases. […] Most patients do not need treatment. Exchange transfusion is occasionally required due to hyperviscosity.

• #24 Polycythemia – StatPearls – NCBI Bookshelf

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

  This is continued weekly until a target hematocrit of under 45% is obtained. […] For secondary polycythemias, phlebotomy is usually reserved for the following conditions: Chronic lung diseases, Cyanotic heart diseases, Post-renal transplant patients with hypertension and erythrocytosis, not responding to optimal doses of angiotensin-converting enzyme inhibitors (ACEIs)/angiotensin receptor blockers (ARBs). […] Hydroxyurea is usually considered second-line therapy. Evidence of benefit came from, among others, a study by the Polycythemia Vera Study Group (PVSG) that showed lower rates of thrombosis compared to a historical cohort treated with phlebotomy alone. […] Indications for use include: Poor venous access, High phlebotomy requirement, When phlebotomy is not possible due to logistic reasons, Severe thrombocytosis, Intractable pruritus.

• #25 Polycythemia – StatPearls – NCBI Bookshelf

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

  This is continued weekly until a target hematocrit of under 45% is obtained. […] For secondary polycythemias, phlebotomy is usually reserved for the following conditions: Chronic lung diseases, Cyanotic heart diseases, Post-renal transplant patients with hypertension and erythrocytosis, not responding to optimal doses of angiotensin-converting enzyme inhibitors (ACEIs)/angiotensin receptor blockers (ARBs). […] Hydroxyurea is usually considered second-line therapy. Evidence of benefit came from, among others, a study by the Polycythemia Vera Study Group (PVSG) that showed lower rates of thrombosis compared to a historical cohort treated with phlebotomy alone. […] Indications for use include: Poor venous access, High phlebotomy requirement, When phlebotomy is not possible due to logistic reasons, Severe thrombocytosis, Intractable pruritus.

• #26 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  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. […] Interferon-alfa is the drug of choice for cytoreductive therapy if needed.

• #27 Polycythemia Vera: A Comprehensive Approach to Management – Hematology Advisor

  https://www.hematologyadvisor.com/features/polycythemia-vera-how-to-treat-approach-management-risk/

  In general, the first-line treatment of choice for cytoreductive therapy for PV is hydroxyurea in conjunction with therapeutic phlebotomy to maintain a goal hematocrit of 45% based on the CYTO-PV study, which demonstrated a reduced rate of cardiovascular events as compared to less-stringent hematocrit control (45%-50%). […] Pegylated interferon alfa is a reasonable first-line alternative to hydroxyurea and may be preferred in very young patients. […] In these special populations, pegylated interferon alfa-2a is a reasonable safer PV treatment option. […] Evidence-based treatment strategies are recommended to maximize life expectancy. […] The potential for combination therapy with ruxolitinib and pegylated interferon alfa-2a is also being explored. […] New treatment options are in development to address the current unmet needs in PV, including ropeginterferon and givinostat, both in phase 3 clinical development.

• #28 How is Polycythemia Vera Treated? | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/how-polycythemia-vera-treated

  Hydroxyurea is a medicine generally used to treat cancer. This medicine can reduce the number of red blood cells and platelets in your blood. As a result, this medicine helps improve your blood flow and bring the thickness of your blood closer to normal. […] Interferon-alpha can prompt your immune system to fight overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Radiation treatment can help suppress overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal. […] Aspirin can relieve bone pain and burning feelings in your hands or feet that you may have as a result of PV. Aspirin also thins your blood, so it reduces the risk of blood clots.

• #29 https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/treatment/special-considerations

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

  Patients with PV have increased risk for bleeding complications after surgery. […] For elective surgeries, it is recommended that your platelet and red blood counts be in normal range before the surgery occurs. […] Anticoagulant therapy should likewise be suspended prior to surgery and restarted after surgery, again when considered acceptable depending on bleeding risk. […] If you have PV and are considering a pregnancy, it is recommended that you meet with an obstetrician specializing in high-risk pregnancies before you become pregnant. […] Your doctor may recommend taking aspirin during and shortly after your pregnancy. […] Hydroxyurea should not be taken during pregnancy or while breastfeeding, as it may harm your baby. […] If you are taking hydroxyurea, you may switch to interferon during your pregnancy.

• #30 Current and future treatment options for polycythemia vera

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4420843/

  Given that not all patients with PV present with splenomegaly, ruxolitinib is also being evaluated in the RESPONSE 2 trial. […] Another class of targeted therapy being explored is histone deacetylase (HDAC) inhibitors, which inhibit proliferation of tumor cells by inducing cell cycle arrest, differentiation, and/or apoptosis. […] Newer pegylated formulations of IFN (PEG-IFN), which are better tolerated and allow for less frequent administration, have renewed interest in IFN as a therapeutic option for patients with PV, including those who are refractory or resistant to HU. […] PV is the most common of the MPNs. Patients experience debilitating pruritus and fatigue and develop new symptoms, such as splenomegaly, as the disease progresses, while facing the risk of major thrombotic events. Although PV is associated with increased mortality, many patients have a long median survival, highlighting the importance of effective and well-tolerated therapy.

• #31 Polycythemia vera – Wikipedia

  https://en.wikipedia.org/wiki/Polycythemia_vera

  Ropeginterferon alfa-2b (Besremi) was approved for medical use in the European Union in February 2019, and in the United States in November 2021. Ropeginterferon alfa-2b is the first medication approved by the U.S. Food and Drug Administration (FDA) to treat polycythemia vera that people can take regardless of their treatment history, and the first interferon therapy specifically approved for polycythemia vera.

• #32 Polycythemia Vera Treatment & Management: Approach Considerations, Medical Care, Phlebotomy

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

  The goals of treatment of polycythemia vera (PV) are as follows: […] The optimal management remains elusive despite the findings of the Polycythemia Vera Study Group (PVSG). However, general principles in the management of PV include the following: […] Normalize red blood cell mass with phlebotomy as rapidly as clinically possible (250-500 mL every other day); patients who are elderly or have cardiovascular compromise should be phlebotomized cautiously, and smaller amounts should be removed. […] The JAK1/2 inhibitor ruxolitinib is approved by the US Food and Drug Administration (FDA) for treatment of PV in patients who have had an inadequate response to or are intolerant of hydroxyurea. […] Ropeginterferon alfa-2b-njft (Besremi) is approved for treatment of adults with PV. It is the first FDA-approved medication for PV that patients can take regardless of their treatment history, and the first interferon therapy specifically approved for PV.

• #33 Treatment Armamentarium for Polycythemia Vera

  https://www.onclive.com/view/treatment-armamentarium-for-polycythemia-vera

  All patients with polycythemia vera should be on a low-dose aspirin, with the exception of those who have insensitivity or, for example, have acquired von Willebrand syndrome. […] The data behind the use of aspirin comes from many older studies. […] The drug of choice for many years and decades has actually been hydroxyurea. […] Overall, hydroxyurea is one of the most commonly used cytoreductive agents in those who need it. […] There is data to support that it more specifically targets the myeloid clonal population. […] The more recent FDA approval in November 2021 of BESREMi, which is a ropeginterferon alpha-2b product. […] Now, after 7.5 years of treatment, in the PEGINVERA study ropeginterferon alpha-2b was able to induce complete hematological responses in 61% of patients. […] Data from PROUD-PV and CONTINUATION-PV is also very encouraging. […] The most striking data is actually about the molecular responses over there. […] The median JAK2 V617F allele burden, which is a marker of the disease, continued to decline on a continuous basis, steadily from baseline to the study follow-up time period.

• #34 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 the condition causes itching, medicines such as antihistamines or treatments with ultraviolet light might give you relief. […] 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.

• #35 Polycythemia Vera Treatment & Management: Approach Considerations, Medical Care, Phlebotomy

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

  Ruxolitinib (Jakafi), a Janus-associated kinase (JAK1/JAK2) inhibitor, was approved by the FDA in 2014 for the treatment of patients with polycythemia vera who have had an inadequate response to or are intolerant of hydroxyurea. […] Another JAK inhibitor, fedratinib (Inrebic), was approved in 2019 for adults with intermediate-2 or high-risk primary or secondary (post-polycythemia vera or post-essential thrombocythemia) myelofibrosis (MF). […] Phlebotomy (bloodletting) has long been the mainstay of therapy for polycythemia vera (PV). […] Once the patient’s hemoglobin and hematocrit values are reduced to within the reference range, implement a maintenance program either by inducing iron deficiency by continuous phlebotomies or by using a myelosuppressive agent. […] The Polycythemia Vera Study Group (PVSG) demonstrated a decreased survival rate and increased mortality rate from acute leukemia in the first 5 years, and a total of 17% of patients had leukemia after 15 years with chlorambucil and with phosphorus-32. […] HU has been the mainstay therapy for PV since the PVSG results indicated it is an effective agent for myelosuppression. […] Low-dose aspirin suppresses thromboxane biosynthesis by platelets, which is increased in PV and essential thrombocythemia.

• #36 Polycythemia Vera Treatment – What Is Jakafi® (ruxolitinib) | Jakafi.com

  https://www.jakafi.com/polycythemia-vera/pv-treatment

  Jakafi (ruxolitinib) is a prescription medicine available as a pill. It is used to treat adults with polycythemia vera (PV) who have already taken a medicine called hydroxyurea and it did not work well enough or they could not tolerate it. […] Jakafi is the first medicine approved by the Food and Drug Administration (FDA) for the treatment of these patients. […] Jakafi is a targeted therapy that works by targeting JAKs, which control the production of blood cells. In doing so, Jakafi helps to reduce overactive JAK signaling to help keep the production of blood cells under control. […] Jakafi is used to treat adults with polycythemia vera who have already taken a medicine called hydroxyurea and it did not work well enough or they could not tolerate it.

• #37 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. […] 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. […] In addition, participants reported reduced severity of their symptoms, such as fatigue, night sweats, problems concentrating, and itching. […] The most common side effect of rusfertide was a reaction at the injection site, Dr. Kremyanskaya said. […] 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. […] 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.

• #38 Current and future treatment options for polycythemia vera

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4420843/

  Low-risk patients (aged60 years and with no prior history of thrombosis) are treated with low-dose aspirin and phlebotomy. […] For high-risk patients, HU or interferon alpha (IFN-) are the first-line treatment recommendations; however, IFN- is not licensed for treatment of PV in most European countries. […] Although some patients respond well to current therapies, effective and well-tolerated treatments are still lacking for both low- and high-risk patients. […] The discovery of JAK2 V617F as the underlying mutation in PV has led to the development of several molecularly targeted therapies focusing on the inhibition of JAK2. […] The first reported results of a JAK2 inhibitor for the treatment of PV were those from the phase 2 study of lestaurtinib in patients with PV/ET. […] In 2011, ruxolitinib, a potent JAK1/JAK2 inhibitor, was approved by the US Food and Drug Administration (FDA) for the treatment of MF and later by the European Medicines Agency for the treatment of splenomegaly and MF-related symptoms.

• #39 Polycythemia – StatPearls – NCBI Bookshelf

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

  The standard daily doses range from 500 to 1500 mg per day. […] The JAK2 inhibitor ruxolitinib is used when patients are intolerant or unresponsive to hydroxyurea. […] Evidence supporting the use of Ruxolitinib in myeloproliferative disorders came from the COMFORT trials. […] The standard recommended dose for polycythemia vera is 10 mg twice a day. […] Low-dose aspirin is indicated when there is inadequate control of microvascular symptoms after achieving the target hematocrit or in the presence of other cardiovascular risk factors. […] Depending on the severity of pruritus and the clinical response to therapy, therapeutic modalities available for symptomatic relief include antihistamines and selective serotonin reuptake inhibitors (SSRIs). […] The standard therapeutic measures of phlebotomy and low-dose aspirin are appropriate in most cases. […] Most patients do not need treatment. Exchange transfusion is occasionally required due to hyperviscosity.

• #40 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  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. […] 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.

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

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

  Management of polycythemia vera (PV) treatment measures are as follows: […] Phlebotomy to keep hematocrit below 45% […] Aspirin 81 mg daily […] Cytoreductive therapy for patients at high risk for thrombosis […] 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 the following: […] Ropeginterferon alfa 2b […] Busulfan in patients older than 65 years […] Ruxolitinib (Jakafi) […] Fedratinib (Inrebic).

• #42 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  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. […] 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.

• #43 Tests and treatment for polycythaemia vera | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/polycythaemia-vera/tests-treatment

  For a high risk of developing blood clots, you may have treatment to control your blood cell levels. The treatments aim to make the blood counts normal. […] Hydroxycarbamide […] Hydroxycarbamide is a chemotherapy drug known as an anti metabolite. […] Peginterferon alfa 2a […] This is a treatment that helps to reduce the rate at which blood cells are made. […] Ruxolitinib […] Ruxolitinib is a type of targeted cancer drug. […] Busulfan […] You might have a chemotherapy drug called busulfan. […] Allopurinol […] Allopurinol lowers the amount of uric acid in the blood and also helps to reduce the symptoms of gout. […] Anagrelide with other cytoreductive drugs […] You might have anagrelide together with other cytoreductive drugs if your platelet levels are abnormal and other treatments are not working.

• #44 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  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. […] 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.

• #45 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. […] Taking part in a clinical trial may be the best treatment choice for some PV patients. […] Treatment decisions are based on the patient’s risk for clotting complications (thrombosis).

• #46 Polycythemia vera: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000589.htm

  The goal of treatment is to reduce the thickness of the blood and prevent bleeding and clotting problems. […] A method called phlebotomy is used to decrease blood thickness. One unit of blood (about 1 pint, or 1/2 liter) is removed each week until the number of red blood cells drops. The treatment is continued as needed. […] Medicines that may be used include: […] Hydroxyurea to reduce the number of red blood cells made by the bone marrow. This drug may be used when the numbers of other blood cell types are also high. […] Interferon to lower blood counts. […] Anagrelide to lower platelet counts. […] Ruxolitinib (Jakafi) to reduce the number of red blood cells and reduce an enlarged spleen. This drug is prescribed when hydroxyurea and other treatments have failed. […] Taking aspirin to reduce the risk of blood clots may be an option for some people, but aspirin increases the risk for stomach bleeding. […] Ultraviolet-B light therapy can reduce the severe itching some people experience.

• #47 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  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. […] Interferon-alfa is the drug of choice for cytoreductive therapy if needed.

• #48 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. […] Taking part in a clinical trial may be the best treatment choice for some PV patients. […] Treatment decisions are based on the patient’s risk for clotting complications (thrombosis).

• #49 Polycythemia Vera: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17742-polycythemia-vera

  Your healthcare provider may prescribe medications to treat the itching symptoms of advanced PV, including: […] These may be used individually or in combination with each other. Examples include: […] Clinical trials offer people the chance to try new emerging treatments while contributing to medical research. […] A bone marrow transplant may be recommended in some cases. […] If PV continues to progress despite treatment, your healthcare provider will focus on relieving your symptoms. Late-stage PV is often characterized by anemia and an increasingly enlarged spleen. You may be treated with:

• #50 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. […] 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. […] In addition, participants reported reduced severity of their symptoms, such as fatigue, night sweats, problems concentrating, and itching. […] The most common side effect of rusfertide was a reaction at the injection site, Dr. Kremyanskaya said. […] 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. […] 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.

• #51 New Agents Advance Treatment Options for Polycythemia Vera

  https://www.targetedonc.com/view/new-agents-advance-treatment-options-for-polycythemia-vera

  This is quite the development. First, interferon approved for PV is safer and appears to be able to be delivered for much longer, maintaining that response and having potential for that biological modification down the road if we continue to treat patients long term. […] Rusfertide is the hepcidin mimetic, the master iron metabolism regulator. It would decrease the availability of iron for blood making, achieve the iron in the reticle interior system of the body, keeping it in the liver lining, the gastrointestinal tract, in the spleen. […] We are excited about having a prospect to the open phase 3 randomized, placebo controlled, blinded study for possible approval of yet another drug for PV patients in those that need to manage phlebotomies. […] Ropeginterferon is a biological agent in the sense that we have interferon in our own body.

• #52 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.

• #53 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 substantially reduce their reliance on blood draws to avoid serious health problems. […] For decades, the main treatment for polycythemia vera has been regular blood draws, or phlebotomy, to remove excess red blood cells. […] So Dr. Kremyanskaya began studying treatments that specifically suppress red blood cell formation calling it a chemical phlebotomy. […] 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 bottom line, Dr. Kremyanskaya said, is that, beyond phlebotomy, there are actually very few treatment options.

• #54 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. […] 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. […] In addition, participants reported reduced severity of their symptoms, such as fatigue, night sweats, problems concentrating, and itching. […] The most common side effect of rusfertide was a reaction at the injection site, Dr. Kremyanskaya said. […] 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. […] 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.

• #55 Current and future treatment options for polycythemia vera

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4420843/

  Given that not all patients with PV present with splenomegaly, ruxolitinib is also being evaluated in the RESPONSE 2 trial. […] Another class of targeted therapy being explored is histone deacetylase (HDAC) inhibitors, which inhibit proliferation of tumor cells by inducing cell cycle arrest, differentiation, and/or apoptosis. […] Newer pegylated formulations of IFN (PEG-IFN), which are better tolerated and allow for less frequent administration, have renewed interest in IFN as a therapeutic option for patients with PV, including those who are refractory or resistant to HU. […] PV is the most common of the MPNs. Patients experience debilitating pruritus and fatigue and develop new symptoms, such as splenomegaly, as the disease progresses, while facing the risk of major thrombotic events. Although PV is associated with increased mortality, many patients have a long median survival, highlighting the importance of effective and well-tolerated therapy.

• #56 New Perspectives on Polycythemia Vera: From Diagnosis to Therapy

  https://www.mdpi.com/1422-0067/21/16/5805

  The use of the JAK1/2 inhibitor ruxolitinib in PV was approved by the FDA in 2014 for patients with an inadequate response to or an unacceptable toxicity from HU. […] For several decades, IFN-alpha has been used to control erythrocytosis in most patients with PV. […] Ropeginterferon alpha-2b (BESREMI, Pharma Essentia) was approved by EMA on February 2019 as monotherapy for the treatment of PV patients without symptomatic splenomegaly. […] Givinostat (Italfarmaco), an HDAC inhibitor, was initially evaluated “in vitro” on JAK2V617F-positive cells from PV and ET patients as well as on the erythroleukemia cell line HEL. […] The murine double minute 2 (MDM2) is a negative regulator of p53, a tumor suppressor protein frequently inactivated in human cancers that controls crucial pathways protecting cells from malignant transformation.

• #57 Polycythemia Vera

  https://www.texasoncology.com/types-of-cancer/myeloproliferative-neoplasms-mpn/polycythemia-vera

  JAK inhibitors: Targeted drugs known as JAK inhibitors may change the way in which myeloproliferative neoplasms are treated. These drugs target abnormal cell signaling that is through to contribute to the growth of myeloproliferative neoplasms. The JAK1 and JAK2 inhibitor Jakafi (ruxolitinib) has been approved for the treatment of myelofibrosis, and is also being studied in Phase III clinical trials for the treatment of PV. […] HDAC inhibitors: Other targeted drugs that are being evaluated for PV include histone deacetylase (HDAC) inhibitors. These drugswhich include givinostat, vorinistat, pabinostat, and othersinterfere with enzymes that may contribute to cancer growth.

• #58 Polycythaemia Vera: Causes, Symptoms, and Treatment

  https://patient.info/doctor/polycythaemia-vera-pro

  Polycythaemia vera treatment and management […] The cornerstone of therapy of low-risk patients remains strict control of cardiovascular risk factors, the use of phlebotomy and low-dose aspirin. Higher risk patients should also receive cytoreductive treatments. Hydroxycarbamide and interferon alfa represent standard first-line options for newly diagnosed high-risk PV patients. […] For patients who fail first-line cytoreductive therapy (hydroxyurea and/or interferon), existing treatment options include alkylating agents, (eg, busulfan, chlorambucil or pipobroman) and P-32. However, there is evidence that these agents are associated with an increased incidence of leukaemic transformation in patients with PV. […] The discovery of mutations in JAK2 as the underlying molecular basis of PV has led to the development of several targeted therapies, including JAK inhibitors – eg, ruxolitinib, which is a selective inhibitor of the Janus-associated tyrosine kinases JAK1 and JAK2 and is licensed in the UK for the treatment of disease-related splenomegaly or symptoms in patients with primary myelofibrosis, post-PV myelofibrosis, or post-essential thrombocythaemia myelofibrosis.

• #59 Current and future treatment options for polycythemia vera

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4420843/

  Low-risk patients (aged60 years and with no prior history of thrombosis) are treated with low-dose aspirin and phlebotomy. […] For high-risk patients, HU or interferon alpha (IFN-) are the first-line treatment recommendations; however, IFN- is not licensed for treatment of PV in most European countries. […] Although some patients respond well to current therapies, effective and well-tolerated treatments are still lacking for both low- and high-risk patients. […] The discovery of JAK2 V617F as the underlying mutation in PV has led to the development of several molecularly targeted therapies focusing on the inhibition of JAK2. […] The first reported results of a JAK2 inhibitor for the treatment of PV were those from the phase 2 study of lestaurtinib in patients with PV/ET. […] In 2011, ruxolitinib, a potent JAK1/JAK2 inhibitor, was approved by the US Food and Drug Administration (FDA) for the treatment of MF and later by the European Medicines Agency for the treatment of splenomegaly and MF-related symptoms.

• #60 Tests and treatment for polycythaemia vera | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/polycythaemia-vera/tests-treatment

  Treatment for PV aims to reduce the number of red blood cells. And reduce the risk of developing more serious problems such as blood clots. […] The treatment you have is individual. Your healthcare team works out if you have a low risk or a high risk of developing a blood clot. This helps them decide the best treatment for you. […] Treatment if you are at low risk for blood clots […] Venesection […] This is a simple procedure that involves removing some of your blood. It is sometimes called phlebotomy. This helps to keep the haematocrit levels low. […] Low dose aspirin […] PV can raise the number of platelets in your blood. This can cause problems with blood clots. Low dose aspirin can lower your risk of getting a blood clot. […] Treatment if you are at high risk for blood clots

• #61 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/

  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. […] 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.

• #62 Polycythemia vera: historical oversights, diagnostic details, and therapeutic views | Leukemia

  https://www.nature.com/articles/s41375-021-01401-3

  Polycythemia vera (PV) is a relatively indolent myeloid neoplasm with median survival that exceeds 35 years in young patients, but its natural history might be interrupted by thrombotic, fibrotic, or leukemic events, with respective 20-year rates of 26%, 16%, and 4%. Current treatment strategies in PV have not been shown to prolong survival or lessen the risk of leukemic or fibrotic progression and instead are directed at preventing thrombotic complications. […] All patients require phlebotomy to keep hematocrit below 45% and once-daily low-dose aspirin, in the absence of contraindications. Cytoreductive therapy is recommended for high-risk or symptomatic low-risk disease; our first-line drug of choice in this regard is hydroxyurea but we consider pegylated interferon as an alternative in certain situations, including in young women of reproductive age, in patients manifesting intolerance or resistance to hydroxyurea therapy, and in situations where treatment is indicated for curbing phlebotomy requirement rather than preventing thrombosis.

• #63 Polycythaemia Vera: Causes, Symptoms, and Treatment

  https://patient.info/doctor/polycythaemia-vera-pro

  Intermittent long-term phlebotomy to maintain the haematocrit below 45% (lower target level may be appropriate for women). Phlebotomy may cause progressive and sometimes severe thrombocytosis and iron deficiency. Splenomegaly and pruritus may persist despite control of the haematocrit by phlebotomy. […] Low-dose aspirin produces a small reduction in thrombotic events, including myocardial infarction and stroke, whilst not increasing the risk of haemorrhage. […] If it is not possible to control thrombotic events with phlebotomy alone then myelosuppression must be considered. However, this is not without risk and increases the risk of leukaemic transformation. Risks and benefits have to be balanced. […] Cytoreductive therapy is recommended for people at high risk of thrombosis (aged over 60 years, or history of thrombosis).

• #64 Tests and treatment for polycythaemia vera | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/polycythaemia-vera/tests-treatment

  Alongside the treatments listed above you might also have: low dose aspirin to lower the risk of blood clots, venesection to reduce the number of red cells in the blood. […] Treatment during pregnancy […] There is a risk of complications including blood clots during pregnancy if you have PV. […] Healthy lifestyle […] Your healthcare team might discuss ways to maintain a healthy lifestyle and help with stopping smoking if you need it.

• #65 Polycythemia Vera: Rapid Evidence Review | AAFP

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

  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. […] Interferon-alfa is the drug of choice for cytoreductive therapy if needed.

• #66 Polycythaemia vera (PV) treatment | Blood Cancer UK

  https://bloodcancer.org.uk/understanding-blood-cancer/polycythaemia-vera-pv/pv-treatment-side-effects/pv-treatment/

  Some drug treatments for PV aim to keep the number of red blood cells in your blood at a stable, low level. […] Busulfan is only used when other drugs have stopped working or arent suitable for you. […] Hydroxycarbamide is a drug you can take as a capsule at home. […] The type of interferon used to treat PV is called interferon alpha. […] Ruxolitinib is a targeted drug called a JAK2 inhibitor. […] Clinical trials are research projects that test new drugs or combinations of drugs on people. […] There is generally a higher risk of thrombosis (blood clots) during pregnancy, so pregnant women with PV need close monitoring. […] It is usually safe to carry on taking aspirin through pregnancy. You may also take an anti-clotting drug called LMWH (low molecular weight heparin).

• #67 https://www.lls.org/myeloproliferative-neoplasms/polycythemia-vera/treatment/special-considerations

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

  Patients with PV have increased risk for bleeding complications after surgery. […] For elective surgeries, it is recommended that your platelet and red blood counts be in normal range before the surgery occurs. […] Anticoagulant therapy should likewise be suspended prior to surgery and restarted after surgery, again when considered acceptable depending on bleeding risk. […] If you have PV and are considering a pregnancy, it is recommended that you meet with an obstetrician specializing in high-risk pregnancies before you become pregnant. […] Your doctor may recommend taking aspirin during and shortly after your pregnancy. […] Hydroxyurea should not be taken during pregnancy or while breastfeeding, as it may harm your baby. […] If you are taking hydroxyurea, you may switch to interferon during your pregnancy.

• #68 Polycythemia vera | University of Iowa Health Care

  https://uihc.org/health-topics/polycythemia-vera

  Your doctor might prescribe additional medication to combat the symptoms of PV. To control itching, you may be given cholestyramine, cyproheptadine, cimetidine, or psoralen plus ultraviolet light treatment. You might also be given medication to counter the symptoms of high uric acid levels in the blood or swelling in the toes. Your doctor is the best person to tell you which treatment is best for you. […] PV requires regular appointments with your doctor. He or she will want to discuss your symptoms and do periodic blood counts to evaluate your response to treatment. […] Polycythemia Vera is a disorder that cannot be cured, but it can be controlled with treatment. Some patients may only need minimal care and observation, while others may require more intensive treatment. In a small number of patients, PV may transform into another type of disorder, especially myelofibrosis or acute leukemia.

• #69 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 the condition causes itching, medicines such as antihistamines or treatments with ultraviolet light might give you relief. […] 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.

• #70 Polycythemia vera | University of Iowa Health Care

  https://uihc.org/health-topics/polycythemia-vera

  Your doctor might prescribe additional medication to combat the symptoms of PV. To control itching, you may be given cholestyramine, cyproheptadine, cimetidine, or psoralen plus ultraviolet light treatment. You might also be given medication to counter the symptoms of high uric acid levels in the blood or swelling in the toes. Your doctor is the best person to tell you which treatment is best for you. […] PV requires regular appointments with your doctor. He or she will want to discuss your symptoms and do periodic blood counts to evaluate your response to treatment. […] Polycythemia Vera is a disorder that cannot be cured, but it can be controlled with treatment. Some patients may only need minimal care and observation, while others may require more intensive treatment. In a small number of patients, PV may transform into another type of disorder, especially myelofibrosis or acute leukemia.

• #71 Polycythaemia vera (PV) | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/blood-cancer/polycythaemia-vera-pv

  Treatment for polycythaemia vera (PV) is usually given to prevent problems such as blood clots. This is called supportive treatment. Treatment can also be given to control PV by reducing the number of red blood cells (cytoreductive therapy). You will have regular blood tests to check for changes in your condition. […] Before treatment, a team of health professionals work together to plan the treatment they feel is best for you. This team is called a multi-disciplinary team (MDT). […] Most people with PV are treated with aspirin tablets. Aspirin affects the way platelets stick together and helps prevent blood clots. Drugs that prevent clots are called anti-platelet drugs or blood thinners. Aspirin does not affect the number of platelets in the blood. […] Regularly removing about a pint (500ml) of blood is a common treatment for PV. It reduces the number of red blood cells in the blood and makes the blood thinner.

• #72 How is Polycythemia Vera Treated? | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-polycythemia-vera/how-polycythemia-vera-treated

  Polycythemia vera (PV) doesn’t have a cure. However, treatments can help control the disease and its complications. PV is treated with procedures, medicines, and other methods. You may need one or more treatments to manage the disease. […] The goals of treating PV are to control symptoms and reduce the risk of complications, especially heart attack and stroke. To do this, PV treatments reduce the number of red blood cells and the level of hemoglobin (an iron-rich protein) in the blood. This brings the thickness of your blood closer to normal. […] Phlebotomy reduces your red blood cell count and starts to bring your blood thickness closer to normal. […] Your doctor may prescribe medicines to keep your bone marrow from making too many red blood cells. Examples of these medicines include hydroxyurea and interferon-alpha.

• #73 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).

• #74 What Is Polycythemia Vera? – Symptoms & Treatment | familydoctor.org

  https://familydoctor.org/condition/polycythemia-vera/

  Polycythemia vera treatment There is no single treatment for PV. Doctors adjust the treatment for each patient. Treatment may include a phlebotomy. In this treatment, some of your blood is removed to help keep the number of red blood cells down and lower the risk of blood clots. Treatment may also include a medicine that keeps the bone marrow from making too many red blood cells or to help reduce the risk of blood clots. Doctors can also treat the itchy skin and burning pain that you may experience. […] With treatments, people who have the disease can live longer lives, many for a normal lifespan. Treatments and regular monitoring from your doctor are an important part of living with PV.

• #75 Polycythemia vera life expectancy: With treatment and more

  https://www.medicalnewstoday.com/articles/polycythemia-vera-life-expectancy

  Polycythemia vera (PV) is a disorder in which the bone marrow produces too many blood cells, leading to blood clots, bleeding, or an enlarged spleen. PV has a reduced life expectancy due to the possible deadly complications. […] Getting treatment can help reduce the risk of certain complications from PV, including blood clots. As a result, a person will likely lead a longer and healthier life with this disease if they receive treatment. […] Treatment for PV can help relieve symptoms and lower the risk of blood clots. In this way, it also reduces a persons risk of life-threatening complications. […] A European study found that the median survival of untreated symptomatic patients with the disease is 6-18 months, whereas, as U.S. research confirmed, people who do get some kind of treatment, may survive a decade or more.

• #76 Current and future treatment options for polycythemia vera

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4420843/

  Patients with polycythemia vera (PV), a myeloproliferative neoplasm characterized by an elevated red blood cell mass, are at high risk of vascular and thrombotic complications and have reduced quality of life due to a substantial symptom burden that includes pruritus, fatigue, constitutional symptoms, microvascular disturbances, and bleeding. Conventional therapeutic options aim at reducing vascular and thrombotic risk, with low-dose aspirin and phlebotomy as first-line recommendations for patients at low risk of thrombotic events and cytoreductive therapy (usually hydroxyurea or interferon alpha) recommended for high-risk patients. However, long-term effective and well-tolerated treatments are still lacking. […] Therapeutic options in PV are limited and no cure is available. The goal of current therapies is to prevent the occurrence of thrombosis/vascular events and delay transformation to MF or AML.

• #77 Treatment for polycythemia vera: New and more

  https://www.medicalnewstoday.com/articles/latest-treatments-for-polycythemia-vera

  Doctors will likely prescribe one or more medications to help reduce the number of blood cells in the bodies of high risk patients. […] Some common medications used for this include: busulfan (Myleran), hydroxyurea (Hydrea), interferon alfa (Intron A), ruxolitinib (Jakafi). […] Researchers are exploring new treatment options. […] Ruxolitinib is a JAK2 inhibitor showing some promise in the treatment of PV. […] There is currently no cure for PV. However, treatments can help improve quality of life. […] Ongoing medical treatment is important to improving both symptoms and life expectancy. […] Polycythemia vera (PV) treatments currently focus on helping prevent thrombosis (blood clots) and negative complications from them, such as heart attack and stroke. […] Future treatments may help with improving quality of life and increasing life expectancy even further.

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