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
- 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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