Polycythemia

For patient information on Neonatal polycythemia, click here

For patient information on Polycythemia vera, click here

Polycythemia
ICD-10	D45, D75.1
ICD-9	238.4, 289.0, 776.4
DiseasesDB	10295
MeSH	D011086

Polycythemia Microchapters
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Case #1
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Debduti Mukhopadhyay, M.B.B.S[2], Zaida Obeidat, M.D.

Synonyms and keywords: Polycythaemia, erythrocythemia, erythrocytosis, packed cell volume increased, PCV increased, primary familial and congenital polycythemia, PFCP, familial erythrocytosis, hereditary erythrocytosis, congenital erythrocytosis, inherited erythrocytosis

Overview

• Medicines that may be used include:
• Hydroxyurea: it considered as a second-line therapy, it showed lower rates of thrombosis compared to phlebotomy alone based on a study by the PVSG.

Indications for use include poor venous access, high phlebotomy requirement, when phlebotomy is contraindicated or not possible, severe thrombocytosis, and unmanageable pruritus.

• Interferon: reduce blood cell counts. Peg interferon can be used to reduce established splenomegaly but not to normal size.
• Anagrelide: used to treat thrombocytosis.
• Ruxolitinib (JAK1/2 inhibitor): it used when patients are intolerant or unresponsive to hydroxyurea, proved to be effective in PPMF and chronic-phase PV; provided a durable relief in symptoms, blood count control, significant reduction in splenomegaly, and better survival based on COMFORT trials.
• Aspirin: indicated at low dose to prevent thrombosis, when there is insufficient control of microvascular symptoms or other cardiovascular risk factors exist.
• Hypouricemic Agents (Allopurinol and febuxostat): required if there is significant hyperuricemia.

• Management of pruritus: antihistamines and selective serotonin reuptake inhibitors (SSRIs) are usually used to relief the symptoms, narrow band ultraviolet-B phototherapy can also be used to treat pruritus.

Sumptomatic Treatment in Polycythemia Vera

Symptoms	Treatment
Pruritus	H1 and H2 blocking antihistamines (diphenhydramine [Benadryl], cyproheptadine [Periactin], hydroxyzine [Atarax, Vistaril], fexofenadine [Allegra], terfenadine [Seldane]) Paroxetine (Paxil) Oatmeal or starch baths (in lukewarm water) Recombinant interferon alfa-2b (intronA)
Erythromelalgia	Aspirin, 50 to 100 mg daily Myelosuppressive agents

Myelosuppressive Agents for the Treatment of Polycythemia Vera

Agent	Class	Common side effects	Uncommon side effects	Percautions
Hydroxyurea (Hydrea)	Antimetabolite	Anemia, neutropenia, oral ulcers, skin ulcers, hyperpigmentation, nail changes	Leg ulcers, nausea, diarrhea, fever, elevated liver function test results	Renal disease
Recombinant interferon alfa-2b (Intron A)	Myelosuppressive	Influenza-like symptoms, fatigue, anorexia, weight loss, alopecia, headache, nausea, insomnia, body pain	Confusion, depression, autoimmunity, hyperlipidemia	Psychiatric disease, cardiovascular disease
Radioactive phosphorus	Radiopharmaceutica	Anemia, thrombocytopenia, leukopenia, Leukemia may develop after treatment	Diarrhea, fever, nausea, emesis	—
Busulfan (Myleran)	Alkylating agent	Pancytopenia, hyperpigmentation, ovarian suppression	Pulmonary fibrosis, leukemia, seizure, hepatic veno-occlusion	Seizure disorder

Treatment

Polycythemia vera is a chronic myeloproliferative disorder characterized by increased red blood cell mass leading to hyperviscosity of the blood which increases the risk of thrombosis. Thrombosis is the leading cause of morbidity and mortality, the main goal of treatment is to prevent thrombotic events include arterial and venous thrombosis, cerebrovascular accident, deep venous thrombosis, myocardial infarction, peripheral arterial occlusion, and pulmonary infarct. In addition to symptomatic treatment of pruritus and distal extremity erythromelalgia.

• Phlebotomy: the backbone of therapy, treatment with phlebotomy alone associated with a longer median survival compared to the use of chlorambucil or radioactive phosphorous based on a trial conducted by PV study group. Repeated phlebotomies help in cytoreduction and reduce hyper-viscosity in addition to induce a state of iron-deficiency which can help retard red-cell proliferation. Weekly sessions are conducted, by remove 500ml of blood until a target hematocrit of under 45% is obtained. This can lower rates of cardiovascular deaths and major thrombotic episodes in patients kept under this threshold based on a trial conducted in Italy. 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 (ARB)

Medicines that may be used include:

• Hydroxyurea: it considered as a second-line therapy, it showed lower rates of thrombosis compared to phlebotomy alone based on a study by the PVSG.

Indications for use include poor venous access, high phlebotomy requirement, when phlebotomy is contraindicated or not possible, severe thrombocytosis, and unmanageable pruritus.

• Interferon: reduce blood cell counts. Peg interferon can be used to reduce established splenomegaly but not to normal size.
• Anagrelide: used to treat thrombocytosis.
• Ruxolitinib (JAK1/2 inhibitor): it used when patients are intolerant or unresponsive to hydroxyurea, proved to be effective in PPMF and chronic-phase PV; provided a durable relief in symptoms, blood count control, significant reduction in splenomegaly, and better survival based on COMFORT trials.
• Aspirin: indicated at low dose to prevent thrombosis, when there is insufficient control of microvascular symptoms or other cardiovascular risk factors exist.
• Hypouricemic Agents (Allopurinol and febuxostat): required if there is significant hyperuricemia.

• Management of pruritus: antihistamines and selective serotonin reuptake inhibitors (SSRIs) are usually used to relief the symptoms, narrow band ultraviolet-B phototherapy can also be used to treat pruritus.

Case Studies

Case #1

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