Hairy cell leukaemia

Last reviewed: 9 Nov 2022
Last updated: 18 Feb 2022

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • abdominal fullness or discomfort
  • splenomegaly
More key diagnostic factors

Other diagnostic factors

  • weakness and fatigue
  • pallor and petechiae
  • fever
  • recurrent infections
  • hepatomegaly
  • superficial and deep lymphadenopathy
  • neurological findings
  • associated systemic immunological disorders
Other diagnostic factors

Risk factors

  • middle age
  • male sex
  • white ancestry
  • western hemisphere location
  • environmental exposures
  • genetic predisposition
  • Epstein-Barr virus
  • infectious mononucleosis
More risk factors

Diagnostic investigations

1st investigations to order

  • FBC
  • peripheral blood smear
More 1st investigations to order

Investigations to consider

  • bone marrow aspiration or biopsy
  • immunophenotyping
  • flow cytometry
  • CT of abdomen
More investigations to consider

Treatment algorithm

ACUTE

symptomatic: without splenic rupture or massive splenomegaly or marked thrombocytopenia precluding chemotherapy

symptomatic: with splenic rupture or massive splenomegaly or marked thrombocytopenia precluding chemotherapy

ONGOING

asymptomatic disease or minimal residual disease after treatment

Contributors

Authors

Ambuj Kumar, MD, MPH

Professor

USF Health Office of Research

Department of Internal Medicine, College of Medicine

Moffitt Cancer Center & Research Institute, Department of Health Outcomes & Behavior

University of South Florida

Tampa

FL

Disclosures

AK declares that he has no competing interests.

Mohamed A. Kharfan-Dabaja, MD, MBA, FACP

Professor

Division of Hematology-Oncology

Blood and Marrow Transplantation Program

Mayo Clinic

Jacksonville

FL

Disclosures

MKD declares that he has no competing interests.

Acknowledgements

Dr Ambuj Kumar and Dr Mohamed Kharfan-Dabaja would like to gratefully acknowledge Dr Benjamin Djulbegovic, a previous contributor to this topic.

Disclosures

BD declares that he has no competing interests.

Peer reviewers

Daniel Catovsky, MD, FRCP, FRCPath, DSc, FMedSc

Consultant Haemato-Oncologist

Section of Haemato-Oncology

Brookes Lawley Institute of Cancer

Sutton

UK

Disclosures

DC declares that he has no competing interests.

Roger Lyons, MD

Clinical Professor of Medicine

University of Texas Health Science Center San Antonio

San Antonio

TX

Disclosures

RL declares that he has no competing interests.

Rebecca Connor, MD

Chief Fellow

Section of Hematology and Oncology

Department of Internal Medicine

Wake Forest University Baptist Medical Center

Winston-Salem

NC

Disclosures

RC declares that she has no competing interests.

Claire Dearden, BSc, MD, FRCP, FRCPath

Consultant Hematologist

The Royal Marsden Hospital

Sutton

Surrey

UK

Disclosures

CD declares that she has no competing interests.

  • Hairy cell leukaemia images
  • Differentials

    • Chronic lymphocytic leukaemia (CLL)
    • Mantle cell lymphoma
    • Pro-lymphocytic leukaemia
    More Differentials
  • Guidelines

    • NCCN clinical practice guidelines in oncology: hairy cell leukemia
    • Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL-V)
    More Guidelines
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