Summary
Definition
History and exam
Key diagnostic factors
- abdominal fullness or discomfort
- splenomegaly
Other diagnostic factors
- weakness and fatigue
- unexplained weight loss
- bruising and bleeding
- pallor and petechiae
- fever
- recurrent infections
- hepatomegaly
- superficial and deep lymphadenopathy
- neurologic findings
- associated systemic immunologic disorders
Risk factors
- middle age
- male sex
- white ancestry
- western hemisphere location
- environmental exposures
- genetic predisposition
- Epstein-Barr virus
- infectious mononucleosis
Diagnostic tests
1st tests to order
- CBC
- peripheral blood smear
- bone marrow trephine biopsy and aspiration (morphology assessment)
- immunohistochemistry
Tests to consider
- flow cytometry
- molecular analysis (for BRAF V600E mutation or IGHV4-34 rearrangement)
- CT of abdomen
Treatment algorithm
symptomatic: without splenic rupture or massive splenomegaly or marked thrombocytopenia precluding chemotherapy
symptomatic: with splenic rupture or massive splenomegaly or marked thrombocytopenia precluding chemotherapy
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 Haematologist
The Royal Marsden Hospital
Sutton
Surrey
UK
Disclosures
CD declares that she has no competing interests.
Differentials
- Chronic lymphocytic leukemia (CLL)
- Mantle cell lymphoma
- Prolymphocytic leukemia
More DifferentialsGuidelines
- 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)
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