Last reviewed: April 2018
Last updated: July  2016

Summary

Definition

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
Full details

Other diagnostic factors

  • features of portal HTN
  • symptoms of pulmonary HTN
  • joint and bone pain
  • hearing loss
  • bleeding
  • infections
Full details

Risk factors

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

Diagnostic investigations

1st investigations to order

  • FBC 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 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
Full details

Treatment algorithm

ACUTE

asymptomatic

symptomatic: aged <50 years and suitable for bone marrow transplant

symptomatic: aged 50-65 years and suitable for bone marrow transplant

symptomatic: aged >65 years or not suitable for bone marrow transplant

Contributors

Authors VIEW ALL

Jerry L. Spivak

Professor of Medicine and Oncology

Division of Hematology

Johns Hopkins University School of Medicine

Baltimore

MD

Disclosures

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

Sheffield

UK

Disclosures

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

Pavia

Italy

Disclosures

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

NY

Disclosures

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

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