CD3(-MHC RESTRICTED CYTOTOXIC T-LYMPHOCYTES IN 2 FRATERNAL AIDS PATIENTS - A CASE-REPORT(), CD56(+) NON)

We describe two brothers with hemophilia and AIDS, with an unusually l arge percentage of CD3(+), CD56(+) lymphocytes, They experienced no ma jor complications with opportunistic infections (01) and infrequent se condary infections, even though they had nearly 0% CD4 lymphocytes for 3 years, Both patients died in 1991 of progressive cardiomyopathy, Th e patients' lymphocytes were immunophenotyped by flow cytometry and an alyzed for functional cytolytic activity against K562 and HIV infected HUT 78 cell lines, Single color CD4 counts were 2-9% for 4 years. Dua l color CD4 counts at our laboratory demonstrated 0-1% CD4 for the las t 6 months, When CD3(+) lymphocytes were examined, both patient 1 and patient 2 demonstrated a significantly higher proportion and absolute number of CD3(''bright''+), CD56(+) double-positive cells, 47% and 22% , respectively, compared to other HIV-positive children with hemophili a (less than or equal to 2%). Functional studies with the K562 target cell line demonstrated the highest natural killer (NK) lymphocyte acti vity in patient 1 that could not be augmented by in vitro addition of IL-2, whereas patient 2 had no NK activity unless IL-2 was added, Func tional studies with HIV-infected HUT-78 cells demonstrated patient 2 h ad cytolytic activity against HIV-infected cells and patient I had hig h nonspecific cytolytic activity even against uninfected HUT-78 cells, whereas controls had minimal activity to HUT-78 cells or HIV-infected HUT-78 cells, The case report raises a speculative question requiring a larger database, whether the anti-HIV activity and/or unusual clini cal course without typical O.I. of some AIDS patients may be related t o the presence of CD3(''bright''+), CD56(+) lymphocytes of their immun e system, (C) 1994 Wiley-Liss, Inc.