Polycythemia Vera

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Related to Polycythemia Vera: Polycythemia rubra vera

polycythemia vera

[‚päl·i‚sī′thē·mē·ə ′vir·ə]
An absolute increase in all blood cells derived from bone marrow, especially erythrocytes.

Polycythemia Vera


(also erythremia, Vaquez’ disease), a chronic disease of the hematopoietic system first described by the French physician L. H. Vaquez in 1892. Polycythemia vera is characterized by an increase in erythrocyte, leukocyte, and platelet mass and in plasma volume. It is classified as a form of leukemia. The course of the disease is comparatively benign. The principal symptoms are a dark red coloration of the skin, elevated blood pressure, proneness to hemorrhaging and thrombosis, and enlargement of the spleen.

Cytological analysis of bone marrow is an important aid in diagnosis. Treatment includes phlebotomy and the administration of cytostatics.


Vorob’ev, A. I., M. D. Brilliant. Patogenez i terapiia leikozov. Moscow, 1976.
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Diagnosis of polycythaemia is revolutionized as a gain-of-function mutation JAK2V617F was found in more than 90% of the patients with polycythemia vera.
There are three classical Philadelphia negative MPN, polycythemia vera (PV), essential thrombocythaemia (ET) and idiopathic myelofibrosis (IMF)13.
As in other series, other diseases were considered in the differential diagnosis, such as cellulitis or vesico-bullous pyoderma gangrenosum; this last entity has a recognized relation with polycythemia vera (5).
She had been admitted to another hospital in Istanbul about 6 months previously for the same symptoms, and she received a diagnosis of polycythemia vera after her blood counts were analyzed.
Clinical diagnosis of PV is based on revised standard diagnosis criteria by Polycythemia Vera Study Group which were divided into 2 groups.
Childhood polycythemia vera and essential thrombocythemia: does their pathogenesis overlap with that of adult patients?
Patients with JAK2 mutation will also require close monitoring with at least yearly follow-up to assess for the development of overt myeloproliferative diseases such as polycythemia vera.
The 2008 WHO classification of MPNs has added new entities, including chronic myelogenous leukemia, Philadelphia chromosome-positive (CML); chronic neutrophilic leukemia (CNL); polycythemia vera (PV); primary myelofibrosis (PMF); essential thrombocythemia (ET); chronic eosinophilic leukemia, not otherwise specified (CEL/NOS); mastocytosis; and myeloproliferative neoplasm, unclassifiable (MPN-U).
It's now become one of our drugs to treat itch, and is the treatment of choice for itch in polycythemia vera," he said.
Overall, 16 of 20 abdominal venous thrombotic events occurred in women, as did 11 of 12 abdominal venous thrombotic events in polycythemia vera, 6 of 7 cerebrovascular accidents, and 8 of 11 transient ischemic attacks.
Chronic myeloproliferative disorders (CMPDs) are clonal haematopoietic stem cell disorders and include the BCR-ABL negative CMPDs like polycythemia vera (PV), essential thrombocythemia (ET), idiopathic myelofibrosis (IMF) and chronic eosinophilic leukaemia (CEL) (1,2).