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Hairy cell leukemia

Last reviewed: 1 Nov 2024
Last updated: 25 Oct 2024

Summary

Definition

History and exam

Key diagnostic factors

  • abdominal fullness or discomfort
  • splenomegaly
Full details

Other diagnostic factors

  • weakness and fatigue
  • unexplained weight loss
  • bruising and bleeding
  • pallor
  • recurrent infections
  • hepatomegaly
  • lymphadenopathy
  • neurologic findings
  • associated systemic immunologic disorders
Full details

Risk factors

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

Diagnostic tests

1st tests to order

  • CBC with differential
  • peripheral blood smear
  • bone marrow trephine biopsy and aspiration (morphology assessment)
  • immunophenotyping (immunohistochemistry or flow cytometry)
  • comprehensive metabolic panel
  • serum lactate dehydrogenase (LDH)
  • viral serology for hepatitis B and C
Full details

Tests to consider

  • molecular analysis (for BRAF V600E mutation or IGHV4-34 rearrangement)
  • CT chest, abdomen, and pelvis
Full details

Treatment algorithm

ACUTE

no indication(s) for treatment

indication(s) for treatment present: without splenic rupture or massive splenomegaly or marked thrombocytopenia precluding chemotherapy

indication(s) for treatment present: with massive symptomatic splenomegaly or splenic rupture or marked thrombocytopenia precluding chemotherapy

ONGOING

early relapse (<2 years) or refractory disease

late relapse (≥2 years)

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 Haematologist

The Royal Marsden Hospital

Sutton

Surrey

UK

Disclosures

CD declares that she has no competing interests.

  • Hairy cell leukemia images
  • Differentials

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    • Prolymphocytic leukemia
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  • 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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