Immunologic Profiles of Adults with Congenital Bleeding Disorders

Abstract

An immunologic profile may be useful to predict the development of Acquired Immune Deficiency Syndrome (AIDS) in both high risk patient groups including homosexuals, hemophiliacs, Haitians, and users of illicit intravenous narcotics as well as the general population. We evaluated 76 consecutive, apparently healthy, adults with congenital bleeding disorders for serum β-2 microglobulin concentration by competitive enzyme immunoassay, T-lymphocyte subpopulations with monoclonal antibodies and serum interferon by inhibition of vesicular stomatitis virus plaque forming units. Findings on physical examination were remarkable with 24% of the group having longstanding splenomegaly and 24% lymphadenopathy. β-2 microglobulin levels were 3232 ± 220 μg/1 (mean ± SEM) with normal controls 2134 ± 119 μg/1. The ratio of Leu_3a (helper/inducer) positive to Leu_2a (suppressor/cytotoxic) positive T-lymphocytes was 1.33 ± 0.1 (mean ± SEM, median = 1.18). Normal control ratios were all >1.35 with a mean ± s.d. = 1.96 ± 0.28. Abnormal ratios of T-lymphocyte subpopulations appeared to persist over time. Increases in β-2 microglobulin correlated with an inverted helper/suppressor T-lymphocyte ratio, the presence of lymphadenopathy, and elevations in aspartate aminotransferase. Interferon was detected in 18% of patient sera. More frequently transfused and more severely affected patients had a higher frequency of immunologic abnormalities although abnormalities also occurred in some rarely and never transfused less severely affected patients. These studies document a high incidence of immunologic abnormalities in patients with inherited coagulation defects.