Summary
Definition
History and exam
Key diagnostic factors
- erythromelalgia
- splenomegaly
- arterial and venous thrombosis
- bleeding
- livedo reticularis
Other diagnostic factors
- age 50 to 70 years
- female gender
- no symptoms
- headache
- dizziness, lightheadedness, and paresthesias
- syncope and seizures
- transient visual disturbances
- hepatomegaly
- priapism
Risk factors
- Unknown risk factors
- Genetic mutations (e.g., Janus kinase 2 [JAK2], calreticulin [CALR], or myeloproliferative leukemia virus oncogene [MPL])
Diagnostic investigations
Investigations to consider
- CRP
- ESR
- fibrinogen
- bone marrow biopsy and histopathology
- Janus kinase 2 (JAK2) mutation testing of peripheral blood
- myeloproliferative leukemia virus oncogene (MPL) mutation testing of peripheral blood
- calreticulin (CALR) mutation testing of peripheral blood
- BCR-ABL fluorescent in situ hybridization (FISH) of bone marrow
Treatment algorithm
Contributors
Authors

Chairman, Department of Internal Medicine
Chief, Cardiovascular Medicine
Professor of Internal Medicine
Texas Tech University
El Paso
TX
Disclosures
DM declares that he has no competing interests.
Peer reviewers
Senior Staff Physician
Henry Ford Medical Center
Sterling Heights
MI
Disclosures
TK declares that he has no competing interests.
Department of Cardiology
Tel Aviv Sourasky Medical Center
Tel Aviv
Israel
Disclosures
UR declares that he has no competing interests.
Professor
Division of Hematology
University of Washington Seattle
WA
Disclosures
DAG declares that he has no competing interests.
Fellow
Division of Hematology
University of Washington
Seattle
WA
Disclosures
BS declares that she has no competing interests.
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