Evaluation of splenectomy in large granular lymphocyte leukaemia.

We performed splenectomy in four patients with severe neutropenia (less than 0.5 X 10(9)/l), recurrent infections, and splenomegaly associated with large granular lymphocyte leukaemia. Prior to splenectomy, elevated levels of neutrophil-reactive IgG were detected in sera of all three patients tested. In all patients, enlargement of the spleen was due to a characteristic lymphoid infiltration of red pulp cords. Splenectomy resulted in an increased neutrophil count greater than 0.5 X 10(9)/l in all patients; this response was sustained in two patients who benefited clinically by a dramatic reduction in frequency of infections. Poor clinical response was associated with elevated levels of antineutrophil antibody post-splenectomy. All four patients had an increase in number of circulating large granular lymphocytes post-splenectomy; one patient who had attained a sustained neutrophil response died of an accelerated lymphoproliferative disorder 19 months post-splenectomy. We conclude that splenectomy may be of value in correcting severe neutropenia and reducing infections in some patients with large granular lymphocyte leukaemia. However, splenectomy appeared to be of no value in treatment of the underlying lymphoproliferative disorder.