title: Spectrum of HTLV-III infection in a hemophilic cohort treated with blood products from a single manufacturer.
abstract: One hundred fifty-eight hemophilia A, B, and von Willebrand disease (VWD) patients treated with clotting factor concentrates from a single manufacturer were tested for antibody to the human T-lymphotropic virus type III (HTLV-III). Antibody was detected in 63% and 40% of those with severe hemophilia A and B, respectively, 12% and 0% of those with mild hemophilia A and B, and two patients with recessive VWD. Forty-two antibody-positive and 20 antibody-negative patients were studied for clinical and laboratory features of infection. Eleven seropositive patients had clinical signs of infection including Pneumocystis carinii pneumonia, lymphadenopathy, splenomegaly or diarrhea; however, only one patient had developed acquired immune deficiency syndrome (AIDS), and only two had significant impairment of their performance status. Thirty-one patients remained totally asymptomatic. Eight patients had a history suggestive of acute HTLV-III infection. Thrombocytopenia was observed in 18% of seropositive patients, lymphopenia in 60%, depressed T-helper cells in 43%, reduced T-helper:T-suppressor ratios (TH:TS) in 33%, and elevated platelet-bound immunoglobulin in 53%. The antibody-negative group had normal T-helper cell levels (except one patient) and TH:TS ratios, and normal platelet immunoglobulin levels. Both groups demonstrated a significant elevation of immunoglobulin levels and a high prevalence of antinuclear factor and antismooth muscle antibodies. The mean level of IgG was significantly higher in the antibody-positive group. This study confirms the correlation between HTLV-III infection and reduced T-helper cells in hemophiliacs but demonstrates a low incidence of clinical symptomatology. There was evidence of polyclonal B-cell hyperactivity in the antibody-negative group as well as the seropositive group.
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000150:complications* / Q000276:immunology; D000293:Adolescent; D000328:Adult; D000368:Aged; D000914:Antibodies, Viral / Q000032:analysis; D001323:Autoantibodies / Q000032:analysis; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D017977:Deltaretrovirus / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000150:complications* / Q000628:therapy; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007970:Leukopenia / Q000150:complications; D008297:Male; D008875:Middle Aged; D013601:T-Lymphocytes / Q000276:immunology; D013921:Thrombocytopenia / Q000150:complications; D014842:von Willebrand Diseases / Q000150:complications
chemical_list: D000914:Antibodies, Viral; D001323:Autoantibodies; D007136:Immunoglobulins