Last reviewed: November 2017
Last updated: November  2017

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 sex
  • no symptoms
  • headache
  • dizziness, lightheadedness, and paraesthesias
  • syncope and seizures
  • transient visual disturbances
  • hepatomegaly
  • priapism

Risk factors

  • Unknown risk factors
  • Janus kinase 2 (JAK2) V617F mutation

Diagnostic investigations

1st investigations to order

  • FBC with peripheral smear
  • iron panel
Full details

Investigations to consider

  • CRP
  • ESR
  • fibrinogen
  • bone marrow biopsy and histopathology
  • Janus kinase 2 (JAK2) V617F mutation testing by real-time quantitative reverse transcription PCR (qRT-PCR) of peripheral blood
  • mutations in the MPL gene
  • calreticulin (CALR) mutation analysis
  • bcr-abl fluorescent in situ hybridisation (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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