Immunologic profiles of adults with congenital bleeding disorders.

Important note: Information in this article was accurate in 1986. The state of the art may have changed since the publication date.

Immunologic profiles of adults with congenital bleeding disorders.

AIDS Res. 1983-84;1(3):163-79. Unique Identifier : AIDSLINE MED/86242456
Goldsmith JC; Moseley PL; Monick M; McCormick JJ; Walker DY; Hunninghake GW

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 beta-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. beta-2 microglobulin levels were 3232 +/- 220 micrograms/l (mean +/- SEM) with normal controls 2134 +/- 119 micrograms/l. The ratio of Leu3a (helper/inducer) positive to Leu2a (suppressor/cytotoxic) positive T-lymphocytes was 1.33 +/- 0.1 (mean +/- SEM, median = 1.18). Normal control ratios were all greater than 1.35 with a mean +/- s.d. = 1.96 +/- 0.28. Abnormal ratios of T-lymphocyte subpopulations appeared to persist over time. Increases in beta-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.

Keywords: beta 2-Microglobulin/ANALYSIS Acquired Immunodeficiency Syndrome/*ETIOLOGY Adolescence Adult Aged Blood Coagulation Factors/ADMINISTRATION & DOSAGE Blood Transfusion Disease Susceptibility Female Hemorrhagic Diathesis/BLOOD/COMPLICATIONS/*IMMUNOLOGY Human Immunoglobulins/ANALYSIS Immunologic Deficiency Syndromes/*COMPLICATIONS Interferons/BLOOD Male Middle Age Support, Non-U.S. Gov't Support, U.S. Gov't, Non-P.H.S. Support, U.S. Gov't, P.H.S. T-Lymphocytes/CLASSIFICATION JOURNAL ARTICLE

861030
M86A0308

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