IMMUNOLOGICAL EVALUATION OF PATIENTS WITH HEMOPHILIA

AIDS is characterized by defects in the cell mediated immune system resulting in unusual infections, neoplasms and immune phenomena. Patients with hemophilia A (HemA) treated with lyophilized commercial FVIII (Lyoph-c) are at risk of developing AIDS since many have AIDS like defects. We have studied 41 patients with hemophilia and Von Willebrand's disease (VWD): 8 Hem A treated with Lyoph-c, 7 Hem A treated with lyophilized volunteer plasma FVIII (lyoph-v), 8 Hem A with inhibitor (Hem A-inh)and 10 Hem B treated with activated or nonactivated prothrombin complex (PTC a/n) and 8 untreated Hem A and VWD. Overall 41% had T helper/T supressor (OK T4/T8) ratios less than 1.4. Although this defect was most marked in the lyoph-c and lyoph-v group (8/15 abnormal), a similar defect was observed in Hem A-inh (3/8), Hem B (2/10) and untreated (4/8) groups. Only two patients with abnormal T4/T8 had laboratory evidence of ongoing viral activity. Serum Beta-2-microglobulin (β2m) levels were elevated (greater than 2000 μg /L) in 93% of treated patients and in only 12.5% of untreated patients. Lymphocyte blastogenesis and MLC were normal in most of the patients. There was no correlation between Tcell markers (T4/T8;T8%;T4%) and amount of factor used. Our results indicate that immunoiogic abnormalities are present in hemophiliacs & VWD,and that all such patients may be at risk for developing AIDS. Elevated β2m in treated group may reflect chronic antigenic stimulation in these patients.