Hairy cell leukaemia. Clinical features and effect of splenectomy.

Abstract

Hairy-cell leukaemia (leukaemic reticuloendotheliosis) is a well-defined clinical entity. Most of the recent reports are almost entirely concerned with the pathological and functional aspects of the disease. In the present retrospective study the clinical features and laboratory data of 12 patients were analyzed together with a series of 123 adequately clinically documented cases from the literature. The Hb level and the sex of the patient proved to be the only parameters having some prognostic value for the survival time after diagnosis. The effect of splenectomy was assessed in two comparable groups of 24 splenectomized and 51 non-splenectomized patients. The operation seemed to be beneficial, but after 2 years the difference was not significant(.05 less than P less than .10). Analysis of subgroups showed that splenectomy was definitely beneficial in women, in patients with a Hb level over 8.0 g/dl or a platelet level above 50 X 10(9)/1, in patients with leucocytes below 3 X 10(9/1, , and also in patients with hepatomegaly (P less than .05 in all cases). These findings suggest that splenectomy is beneficial in cases where anaemia and thrombocytopenia are not very severe; in severe cytopenia the operation does not increase the life expectancy.