Polycythemia vera

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Polycythemia vera
ICD-10	D 45.
ICD-9	238.4
ICD-O:	9950/3
MeSH	D011087


Polycythemia vera Microchapters
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Pathophysiology

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Differentiating Polycythemia vera from other Diseases

Epidemiology and Demographics

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Case #1

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

Synonyms and keywords: Osler-Vaquez disease; Vaques-Osler arythremia; Vaquez' polycythaemia; Vaquez-Osler disease;

Overview

Historical Perspective

Classification

Pathophysiology

Differentiating Polycythemia vera from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Treatment

Case Studies

Case #1

v • d • e

Pathology: hematology, myeloid hematologic disease (primarily D50-D77, 280-289)

RBCs/
hemoglobinopathy

Polycythemia - Macrocytosis

Anemia

Nutritional

Iron deficiency anemia (Plummer-Vinson syndrome), Megaloblastic anemia (Pernicious anemia)

Hemolytic

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

Aplastic

Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia, Sideroblastic anemia

Blood tests

Normocytic - Microcytic - Macrocytic - Normochromic - Hypochromic

Other

Methemoglobinemia

Coagulation/platelets/
coagulopathy/
bleeding diathesis

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

clotting factor: Hemophilia (A/VIII, B/IX, C/XI) • Von Willebrand disease • Hypoprothrombinemia/II - XIII

platelet function: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Gray platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly

Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP

Thrombocytopenia (Heparin-induced thrombocytopenia)

Monocytes/
macrophages

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

-penia: Monocytopenia

Granulocytes

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

See also hematological malignancy and immune disorders

v • d • e

Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208)

CFU-GM/
and other granulocytes

CFU-GM

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-Baso

AML (Acute basophilic)

CFU-Eos

AML (Acute eosinophilic)
MP (Chronic eosinophilic leukemia/Hypereosinophilic syndrome)

MEP

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)