Last reviewed: 22 May 2021
Last updated: 26 Jul 2018



History and exam

Key diagnostic factors

  • history of radiation exposure
  • history of industrial solvents exposure
  • constitutional symptoms (weight loss, night sweats, low-grade fever, cachexia, fatigue, and pruritus)
  • splenomegaly ± hepatomegaly
  • features of extramedullary hematopoiesis

Other diagnostic factors

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

Risk factors

  • radiation exposure
  • industrial solvents exposure
  • age >65 years

Diagnostic investigations

1st investigations to order

  • CBC with differential
  • peripheral blood smear
  • bone marrow aspiration
  • bone marrow biopsy

Investigations to consider

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

Treatment algorithm


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 reviewersVIEW ALL

Professor and Consultant in Haematology

Royal Hallamshire Hospital




JTR is an author of a number of references cited in this topic.

Director of the Laboratory of Clinical Epidemiology

IRCCS Policlinico S. Matteo Foundation




GB declares that he has no competing interests.

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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