Case Studies
Case 6.4 Hairy cell leukaemia
A 48-year-old asymptomatic man was found to have gross splenomegaly on a routine life-insurance examination. Investigation showed a normal haemoglobin but a mildly raised white-cell count (14.2 x 109/l). On the blood film these cells were mainly small mononuclear cells resembling lymphocytes, but had a spiky or 'hairy' appearance, had B-cell markers on their surface and were positive for kappa but not lambda light chains (see Table C6.2). They stained positively for tartrate-resistant acid phosphatase, an enzyme characteristically found in the cells of hairy cell leukaemia (see Fig. 6.5). Bone marrow aspirate was 'dry' and trephine showed a characteristic infiltrate.
Splenectomy is now reserved for those with pancytopenia or splenic infarction. This patient was treated with pentostatin which is the treatment of choice for hairy cell leukaemia. He has remained well for 5 years.
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