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Primary splenic hairy cell leukaemia variant presenting as immune thrombocytopenic purpura

The disease now known as hairy cell leukaemia (HCL) is a chronic B-cell leukaemia accounting for approximately 2% of adult leukaemia. It was first described as a distinct clinical entity by Bouroncle et al. in 1958 (1) and termed leukaemic reticuloendotheliosis. The term hairy cell leukaemia was coined by Schrek & Donnelly in 1966 (2). In 1980 a rare variant of HCL (HCL-V) was first described (3) and subsequently further cases have been reported, the largest series being from The Royal Marsden Hospital (4). Primary splenic HCL is another very rare entity (5-8) suggesting that the malignant cell in HCL may originate in the spleen. However, to date there have been no reported cases of primary splenic HCL-V. We describe a case of apparent primary splenic HCL-V, presenting as immune thrombocytopenic purpura (ITP), in which splenectomy resulted in both clinical and haematological remissions. This adds further support to the hypothesis that the spleen is the site of origin of these disorders.