Hairy cell leukaemia (HCL) is an uncommon, indolent, mature B-cell neoplasm. It is characterised by symptoms of fatigue, a markedly enlarged spleen, and a distinctive histological appearance on peripheral blood smear and bone marrow biopsy.
The disease is not curable. However, it is highly responsive to therapy and may be managed successfully for a decade or more.
Patients who are without symptoms do not require immediate treatment; early treatment does not extend life expectancy.
In symptomatic patients, purine analogues have largely replaced traditional therapies, such as splenectomy, except in rare cases of splenic rupture or massive splenomegaly.
Supportive care with antibiotics, non-steroidal anti-inflammatory drugs, administration of irradiated blood products, and granulocyte colony-stimulating factors may have a role in therapy for HCL.
Hairy cell leukaemia (HCL) is an uncommon, indolent, mature B-cell neoplasm. Seen under the microscope, the cells have delicate cytoplasmic projections resembling hair (‘hairy cells’). The disease has a characteristic presentation of pancytopenia, splenomegaly, and hairy cells in the circulation, marrow, and other haematopoietic organs.
History and exam
Key diagnostic factors
- presence of risk factors
- abdominal fullness or discomfort
Other diagnostic factors
- weakness and fatigue
- pallor and petechiae
- recurrent infections
- superficial and deep lymphadenopathy
- neurological findings
- associated systemic immunological disorders
- middle age
- male sex
- white ancestry
- western hemisphere location
- environmental exposures
- genetic predisposition
- Epstein-Barr virus
- infectious mononucleosis
1st investigations to order
- peripheral blood smear
Investigations to consider
- bone marrow aspiration or biopsy
- flow cytometry
- CT of abdomen
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
Ambuj Kumar, MD, MPH
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
AK declares that he has no competing interests.
Mohamed A. Kharfan-Dabaja, MD, MBA, FACP
Division of Hematology-Oncology
Blood and Marrow Transplantation Program
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.
Daniel Catovsky, MD, FRCP, FRCPath, DSc, FMedSc
Section of Haemato-Oncology
Brookes Lawley Institute of Cancer
DC declares that he has no competing interests.
Roger Lyons, MD
Clinical Professor of Medicine
University of Texas Health Science Center San Antonio
RL declares that he has no competing interests.
Rebecca Connor, MD
Section of Hematology and Oncology
Department of Internal Medicine
Wake Forest University Baptist Medical Center
RC declares that she has no competing interests.
Claire Dearden, BSc, MD, FRCP, FRCPath
The Royal Marsden Hospital
CD declares that she has no competing interests.
- Chronic lymphocytic leukaemia (CLL)
- Mantle cell lymphoma
- Pro-lymphocytic leukaemia
- 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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