Polycythemia vera overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Primary polycythemia, often called polycythemia vera (PCV), polycythemia rubra vera (PRV), or erythremia, occurs when excess red blood cells are produced as a result of an abnormality of the bone marrow. Often, excess white blood cells and platelets are also produced. Polycythemia vera is classified as a myeloproliferative disease.

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Nutritional

Hereditary	enzyme: G6PD Deficiency - Pyruvate kinase deficiency - Triosephosphate isomerase deficiency hemoglobin: Thalassemia - Sickle-cell disease/trait membrane: Hereditary spherocytosis - Hereditary elliptocytosis - Hereditary stomatocytosis
Acquired	Autoimmune (Warm, Cold), HUS, MAHA, PNH, PCH, Myelophthisic

Other

Hypercoagulability	primary: Antithrombin III deficiency - Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden - Hyperprothrombinemia acquired: DIC (Congenital afibrinogenemia, Purpura fulminans) - autoimmune (Antiphospholipid)
Other	Essential thrombocytosis

Histiocytosis	WHO-I (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease)
Other	Chronic granulomatous disease -cytosis: Monocytosis

+	-cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia)
-	-penia: Granulocytopenia/agranulocytosis (Neutropenia/Kostmann syndrome, Eosinopenia, Basopenia)

See also hematological malignancy and immune disorders

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Myelocyte	AML: Acute myeloblastic leukemia (M0, M1, M2), APL/M3 MP (Chronic neutrophilic leukemia)
Monocyte	AML (AMoL/M5, Myeloid dendritic cell leukemia) CML (Philadelphia chromosome, Accelerated phase chronic myelogenous leukemia)
Myelomonocyte	AML (M4) MD-MP (Juvenile myelomonocytic leukemia, Chronic myelomonocytic leukemia)
Other	Histiocytosis

CFU-Meg	AML (AMKL/M7) MP (Essential thrombocytosis)
CFU-E	AML (Erythroleukemia/M6) MP (Polycythemia vera) MD (Refractory anemia, Refractory anemia with excess of blasts, Chromosome 5q deletion syndrome, Sideroblastic anemia, Paroxysmal nocturnal hemoglobinuria, Refractory cytopenia with multilineage dysplasia)