Association of pure red cell aplasia with T large granular lymphocyte leukaemia

To define the relation between T large granular lymphocyte (T-LGL) leukaemia and pure red cell aplasia in Chinese patients.

Patients with T-LGL leukaemia were identified from a consecutive series of Chinese patients with chronic lymphoproliferative disorders. The diagnosis of T-LGL leukaemia was based on typical morphological and immunophenotypical features, and confirmed by the detection of clonal T cell receptor gene rearrangement. The clinicopathological features, response to treatment, and long term follow up were also examined.

Five patients were identified as having T-LGL leukaemia from a consecutive series of 33 Chinese patients with chronic lymphoproliferative disorders. The median follow up time was 45 months. An obvious lymphocytosis was present in only two cases, although an increase in large granular lymphocytes in the peripheral blood was found in four. In one case, the LGL count was within the normal range. Epstein-Barr virus encoded early nuclear RNA was negative in all the cases. There was no evidence of rheumatoid arthritis, and none of the patients presented with recurrent infections. On follow up, pure red cell aplasia occurred at some stage of the disease in all the patients. This responded to treatment with cyclosporin A in two and with antithymocyte globulin in one. Two patients remained transfusion dependent.

In contrast to Western patients, Chinese patients with T-LGL leukaemia do not appear to suffer from rheumatoid arthritis and recurrent infections, but pure red cell aplasia is a major cause of morbidity in this ethnic group.