Polycythemia vera epidemiology and demographics

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

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Overview

Incidence

The overall incidence in the Minnesota population was 1.9 per 100,000 person-years

Age

Polycythemia vera occurs in all age groups (including children),^[1] although the incidence increases with age. One study found the median age at diagnosis to be 60 years,^[2] while a study in Olmsted County, Minnesota found that the highest incidence was in people aged 70–79 years.^[3]

Gender

The disease was more common in men than women.^[3]

References

↑ Passamonti F, Malabarba L, Orlandi E, Baratè C, Canevari A, Brusamolino E, Bonfichi M, Arcaini L, Caberlon S, Pascutto C, Lazzarino M (2003). "Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia.". Haematologica 88 (1): 13-8. PMID 12551821.
↑ Berlin, NI. (1975). "Diagnosis and classification of polycythemias". Semin Hematol 12: 339.
↑ ^3.0 ^3.1 Anía B, Suman V, Sobell J, Codd M, Silverstein M, Melton L (1994). "Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989.". Am J Hematol 47 (2): 89-93. PMID 8092146.

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)