Last reviewed: 6 Jan 2023
Last updated: 26 Jul 2018



History and exam

Key diagnostic factors

  • presence of risk factors
  • constitutional symptoms (weight loss, night sweats, low-grade fever, cachexia, fatigue, and pruritus)
  • splenomegaly ± hepatomegaly
  • features of extramedullary haematopoiesis
More key diagnostic factors

Other diagnostic factors

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

Risk factors

  • radiation exposure
  • industrial solvents exposure
  • age >65 years
More risk factors

Diagnostic investigations

1st investigations to order

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

Investigations to consider

  • genetic mutation analysis (JAK2 V617F, calreticulin [CALR], MPL)
  • breakpoint cluster region-abelson polymerase chain reaction (BCR-ABL PCR)
  • chromosomal assessment in bone marrow examination
  • CD34+ cell count
  • antinuclear antibodies
  • rheumatoid factor titre
  • complement levels
  • Coombs' test
  • echocardiogram
  • ultrasound of suspected site
  • technetium 99 scan
  • CT of suspected site
  • MRI of suspected site
  • serum uric acid
More investigations to consider

Treatment algorithm



symptomatic: aged <50 years and suitable for stem cell transplant

symptomatic: aged ≥50 years and suitable for stem cell transplant

symptomatic: 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 a reference cited in this topic and is a consultant for Incyte.


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 a number of 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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