Acquired-immunodeficiency-like syndrome in two haemophiliacs.
The immunological status of two multiply transfused patients with severe haemophilia A and diffuse lymphadenopathy was evaluated. But patients appeared clinically well and denied homosexuality or intravenous drug abuse. Immunological studies revealed depressed cellular immune function with anergy, relative lymphopenia, decreased T helper cells, and increased T suppressor cells in both patients and an altered T-helper-cell/T-suppressor-cell ratio in one. Hyperresponsiveness of the humoral immune system was demonstrated by elevated IgG and IgM. Lymph-node biopsy revealed benign nonspecific hyperplasia in both patients. The lymphadenopathy and immunological features in these two haemophiliacs bear a striking resemblance to the acquired immunodeficiency syndrome (AIDS) of homosexuals, intravenous drug abusers, and Haitian immigrants. These findings may represent a prodromal phase or a forme fruste of AIDS. Transmission of an infectious agent in blood products seems likely.
D000163:Acquired Immunodeficiency Syndrome / Q000175:diagnosis*; D000293:Adolescent; D000328:Adult; D001706:Biopsy; D003937:Diagnosis, Differential; D006467:Hemophilia A / Q000175:diagnosis / Q000276:immunology*; D006801:Humans; D006965:Hyperplasia; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
D007074:Immunoglobulin G; D007075:Immunoglobulin M