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Last reviewed: 30 Oct 2023
Last updated: 22 Mar 2023



History and exam

Key diagnostic factors

  • history of radiation exposure
  • history of industrial solvents exposure
  • symptoms of anemia (fatigue, weakness, dyspnea, palpitations)
  • constitutional symptoms (weight loss, night sweats, low-grade fever, cachexia, fatigue, and pruritus)
  • splenomegaly ± hepatomegaly
  • features of extramedullary hematopoiesis
More key diagnostic factors

Other diagnostic factors

  • features of portal hypertension
  • joint and bone pain
  • hearing loss
  • bleeding
  • infections
Other diagnostic factors

Risk factors

  • radiation exposure
  • industrial solvents exposure
  • age ≥65 years
  • cytogenetic abnormalities
More risk factors

Diagnostic investigations

1st investigations to order

  • CBC with differential
  • peripheral blood smear
  • bone marrow aspiration
  • bone marrow biopsy
More 1st investigations to order

Investigations to consider

  • genetic mutation analysis (including JAK2 V617F, calreticulin [CALR], MPL)
  • fluorescence in situ hybridization (FISH) or breakpoint cluster region-abelson polymerase chain reaction (PCR)
  • bone marrow cytogenetic analysis
  • echocardiogram
  • ultrasound of suspected site
  • technetium 99 scan
  • CT of suspected site
  • MRI of suspected site
  • serum uric acid
  • antinuclear antibodies
  • rheumatoid factor titer
  • complement levels
  • Coombs test
More investigations to consider

Treatment algorithm


lower risk: asymptomatic

lower risk: symptomatic

higher risk: ages <50 years and suitable for stem cell transplant

higher risk: ages ≥50 years and suitable for stem cell transplant

higher risk: not suitable for stem cell transplant



Jerry L. Spivak, MD
Jerry L. Spivak

Professor of Medicine and Oncology

Division of Hematology

Johns Hopkins University School of Medicine




JLS is an author of several references cited in this topic.


Professor Jerry Spivak would like to gratefully acknowledge Dr Ashkan Emadi, a previous contributor to this topic.


AE declares that he has no competing interests.

Peer reviewers

John T. Reilly, BSc, MD, FRCP, FRCPATH

Professor and Consultant in Haematology

Royal Hallamshire Hospital




JTR is an author of several references cited in this topic.

Giovanni Barosi, MD

Director of the Laboratory of Clinical Epidemiology

IRCCS Policlinico S. Matteo Foundation




GB declares that he has no competing interests.

Richard Silver, MD

Myeloproliferative Disorders Program Specialist

Department of Medicine

Division of Hematology and Medical Oncology

Weill Cornell Medical College

New York



RS is an author of a reference cited in this topic.

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