Natural history

18fl Hairy cell leukaemia is associated with prolonged survival (95% at 5 years), 180 with newer agents capable of producing remissions; some patients may achieve long term cure. For others careful application of available treatments at points of disease relapse/progression will still allow prolonged, good quality survival. If a remission of >5 years has been achieved then a further remission with the same agent is likely. Hairy cell variant

Describes a very rare variant of HCL where the presenting WBC count is high due to circulating leukaemic cells (40-60 x 109/L) and monocy-topenia is absent. Cells are villous but have a central round nucleus and a distinct nucleolus like PLL. Marrow is aspirated easily due to low reticulin but the trephine appearance is similar to HCL and associated neutropenia. Immunophenotype differs from typical HCL: CD11c+, CD25 & HC2 -ve, CD103 usually -ve. Response to deoxycoformycin or IFN-a is poor but chlorambucil appears active in this form, and the variant generally follows an indolent course.

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