Last reviewed: April 2018
Last updated: May  2018

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

1st investigations to order

  • CBC with peripheral smear
  • iron panel
Full details

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
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Debabrata Mukherjee

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 VIEW ALL

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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