Last reviewed: June 2018
Last updated: April  2017



History and exam

Key diagnostic factors

  • hx of radiation exposure
  • hx 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 HTN
  • symptoms of pulmonary HTN
  • 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
Full details

Investigations to consider

  • peripheral blood JAK2 V617F mutation
  • BCR-ABL PCR (breakpoint cluster region-abelson [BCR-ABL] polymerase chain reaction [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
Full details

Treatment algorithm


Authors VIEW ALL

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 monograph and is a consultant for Incyte.

Professor Jerry Spivak would like to gratefully acknowledge Dr Ashkan Emadi, a previous contributor to this monograph. AE declares that he has no competing interests.

Peer reviewers VIEW ALL

Professor and Consultant in Haematology

Royal Hallamshire Hospital




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

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 monograph.

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