title: Acquired immunodeficiency syndrome and other possible immunological disorders in European haemophiliacs.
abstract: A questionnaire concerning the occurrence of acquired immunodeficiency syndrome (AIDS) and possible related clinical syndromes in treated haemophiliacs was sent to directors of 201 European haemophilia centres. 135 replies were received from eighteen countries, covering 8928 patients with haemophilia A, 1889 with haemophilia B, and 2330 with von Willebrand's disease treated with blood products per year; 8 cases of AIDS were identified, one of which was in a patient with haemophilia B; 7 suffered from infections and 1 from pleomorphic non-Hodgkin's lymphoma. 3 other patients with lymphoma were recorded. The median age was 20 years with range 12-57 years and 3 were aged 14 years or less; 3 of the patients had died. 179 patients with other possible immunological disorders were recorded including 86 with lymphadenopathy but 70 of these occurred at one centre. At least 48 patients with thrombocytopenia were identified, suggesting that this condition is more common in treated haemophiliacs than is "idiopathic thrombocytopenic purpura" in the general population. None of the abnormalities could be related to use of any particular type of factor VIII concentrates. These results, together with 3 cases of AIDS previously reported and not returned in this study, point to an incidence of 11 cases in 13 000 treated haemophiliacs.
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000150:complications* / Q000453:epidemiology; D000293:Adolescent; D000328:Adult; D000380:Agranulocytosis / Q000150:complications; D002648:Child; D005060:Europe; D005169:Factor VIII / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications* / Q000628:therapy; D002836:Hemophilia B / Q000150:complications* / Q000628:therapy; D006801:Humans; D007154:Immune System Diseases / Q000150:complications*; D008206:Lymphatic Diseases / Q000150:complications; D008297:Male; D008875:Middle Aged; D011696:Purpura, Thrombocytopenic / Q000150:complications; D006113:United Kingdom; D014842:von Willebrand Diseases / Q000150:complications* / Q000628:therapy
chemical_list: D005169:Factor VIII