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Polycythemia vera.

Brian J Stuart¹, Anthony J Viera

¹Naval Hospital Jacksonville, Family Practice Department, Jacksonville, Florida 32214, USA. stuartbj@2fssg.usmc.mil

American Family Physician

|May 22, 2004

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Polycythemia vera, a blood disorder causing high red blood cell mass, increases thrombosis risk. Early diagnosis and treatment, including phlebotomy and myelosuppressants, significantly improve patient survival and quality of life.

Area of Science:

Hematology
Oncology

Background:

Polycythemia vera is a chronic myeloproliferative neoplasm.
It is characterized by an overproduction of red blood cells, leading to hyperviscosity and thrombosis.
Secondary causes of erythrocytosis must be excluded before diagnosis.

Purpose of the Study:

To outline the diagnostic criteria for Polycythemia vera.
To discuss the clinical presentation and complications.
To review current and investigational treatment strategies.

Main Methods:

Diagnosis relies on criteria from the Polycythemia Vera Study Group.
Key diagnostic indicators include elevated red blood cell mass and splenomegaly.
Exclusion of secondary causes is critical.

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Main Results:

Untreated Polycythemia vera has a poor prognosis (6-18 months survival).
Effective treatment can extend life expectancy to over 10 years.
Phlebotomy is a primary treatment, often combined with myelosuppressive agents.

Conclusions:

Polycythemia vera requires prompt diagnosis and management.
Risk-stratified treatment approaches improve outcomes.
Ongoing research explores novel therapeutic agents like interferon alfa-2b, anagrelide, and aspirin.