Summary
Definition
Hairy cell leukaemia (HCL) is an uncommon, indolent, mature B-cell neoplasm. It was described as a distinct clinical entity in 1958.
[1]
Seen under the microscope, the cells have delicate cytoplasmic projections, resembling hair, and that is why the disease was named HCL. The disease has a characteristic presentation of pancytopenia, splenomegaly, and circulating hairy cells in blood, marrow, and other haematopoietic organs.
[2]
[Figure caption and citation for the preceding image starts]:
Cytospin prepared from bone marrow aspirate illustrates the typical cell cytology, with oval- to bean-shaped nuclei and moderate amounts of cytoplasm with irregular cytoplasmic borders (Wright Giemsa 100x oil)
From the collection of Lynn Moscinski, MD
[Citation ends].
[Figure caption and citation for the preceding image starts]:
Sections of core biopsy demonstrate lymphocytes with obvious cytoplasm within the marrow interstitium, associated with dilatation of marrow sinuses and red blood cell collections (H&E 50x oil)
From the collection of Lynn Moscinski, MD
[Citation ends].
History and exam
Key diagnostic factors
- presence of risk factors
- abdominal fullness or discomfort
- splenomegaly
Other diagnostic factors
- weakness and fatigue
- pallor and petechiae
- fever
- recurrent infections
- hepatomegaly
- superficial and deep lymphadenopathy
- neurological findings
- associated systemic immunological disorders
Risk factors
- middle age
- male sex
- white ancestry
- western hemisphere location
- environmental exposures
- genetic predisposition
- Epstein-Barr virus
- infectious mononucleosis
Diagnostic investigations
Investigations to consider
- bone marrow aspiration or biopsy
- immunophenotyping
- flow cytometry
- tartrate-resistant acid phosphatase (TRAP)
- CT of abdomen
Treatment algorithm
Contributors
Authors VIEW ALL
Associate Professor
USF Center for Comparative Effectiveness Research and Evidence Based Medicine
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.
Professor
Division of Hematology-Oncology
Blood and Marrow Transplantation Program
Mayo Clinic
Jacksonville
FL
Disclosures
MKD declares that he has no competing interests.
Dr Ambuj Kumar and Dr Mohamed Kharfan-Dabaja would like to gratefully acknowledge Dr Benjamin Djulbegovic, a previous contributor to this topic. BD declares that he has no competing interests.
Peer reviewers VIEW ALL
Consultant Haemato-Oncologist
Section of Haemato-Oncology
Brookes Lawley Institute of Cancer
Sutton
UK
Disclosures
DC declares that he has no competing interests.
Clinical Professor of Medicine
University of Texas Health Science Center San Antonio
San Antonio
TX
Disclosures
RL declares that he has no competing interests.
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.
Consultant Hematologist
The Royal Marsden Hospital
Sutton
Surrey
UK
Disclosures
CD declares that she has no competing interests.
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