Persistent lymphadenopathy associated with hypertransfusion in sickle-cell disease

We report the results of an immunologic evaluation of two hypertransfused (HT) patients with sickle-cell disease (SCD) who have developed persistent generalized lymphadenopathy. In order to interpret the results of this evaluation, we studied seven other HT patients with SCD and seven nonhypertransfused patients with SCD. Patients with SCD had decreased percentages of T-lymphocytes to include both helper and suppressor subsets in their peripheral blood. These decreases resulted in T helper/suppressor ratios not different from those of healthy normal control subjects. Lymphocyte proliferative responses to mitogen were decreased in the nonhypertransfused group, whereas mononuclear cell populations from both patient groups had higher levels of spontaneous suppressor cell activity than did control subjects. The patients with lymphadenopathy were distinguished from other HT sickle-cell anemia patients by immunologic abnormalities that included decreased percentages of T4+ lymphocytes, decreased T helper/suppressor ratios, and decreased lymphocyte responses to mitogen. Furthermore, the serum of these patients contained antibody specific for human T cell lymphotropic virus type III (HTLV-III). We believe that these two patients have developed acquired immunodeficiency syndrome-related lymphadenopathy as the result of transfusion-acquired HTLV-III. We propose that hypertransfusion treatment in SCD, possibly in association with phenytoin administration, places individuals at risk for HTLV-III-associated syndromes.