Hairy cell leukaemia (HCL)

Overview:

• HCL is a clonal proliferation of abnormal B (or very rarely T) cells which, as in CLL, accumulate in the bone marrow and spleen
• Is a rare disease of late middle age
• The bizarre name relates to the appearance of the cells on a blood film:
• They have an irregular outline owing to the presence of filament-like cytoplasmic projections

Clinical features:

Symptoms:

• Those of anaemia
• Recurrent infections
• Abdominal discomfort owing to splenic enlargement

Signs:

• Those of anaemia
• A palpable spleen

Investigations:

FBC:

• Pancytopenia

Bone marrow aspirate:

• Shows increased cellularity with characteristic infiltration by ‘hairy’ cells

Treatment:

• 2-chloroadenosine (2-CDA) has been shown to have specific activity in this illness, complete remission often being achieved with just 1 cycle of treatment
• the remissions sometimes last for several years and patients can be re-treated