Biochemical and immunologic abnormalities in peripheral blood T lymphocytes of patients with hemophilia A.

Subset distribution, ecto-5'nucleotidase (5'NT) activity, and the generation of allospecific cytotoxic T lymphocytes (CTL) were investigated in peripheral blood T lymphocytes from 39 hemophilia A patients divided into three groups: group A and group B (HIV-patients with CD4:CD8 ratio less than 1 and greater than 1 respectively), and group C (HIV + patients). 5'NT activity was significantly decreased compared with healthy controls in groups B and C. In group B, this deficiency was attributable to expansion of CD8 + CD11 + suppressor cells. In group C, activated (HLA-DR+) CD8+ cells were also present and contributed to 5'NT deficiency. The suppressor cell expansion seemed to be mainly related to AHF infusions, whereas HLA-DR expression was related to HIV infection. CD11+ and HLA-DR+ cells were also expanded in the CD4+ subpopulation of all three groups, whereas CD4+ CD28+ lymphocytes were significantly decreased in group C only. Lastly, alloreactive cytotoxicity was decreased in group B and was normal in groups A and C.