title: High-dose intravenous immunoglobulin and splenectomy for the treatment of HIV-related immune thrombocytopenia in patients with severe haemophilia.
abstract: Four patients with severe haemophilia A and one patient with severe Christmas disease developed severe immune thrombocytopenia (platelet count less than 20 x 10(9)/l). All five patients were HIV-antibody positive and one was HIV-antigen positive. Four patients were treated initially with prednisolone, but with only a transient platelet response in three and no response in the fourth. All patients were treated with high dose intravenous immunoglobulin (0.4 g/kg daily for 5 d) resulting in a rise in platelet count in all cases (range 138-300 x 10(9)/l) and then proceeded to splenectomy. Three remain in complete remission after 6-14 months, and one showed a good response with platelet counts ranging from 103 to 187 x 10(9)/l. The fifth patients achieved a normal platelet count for 3 months post-splenectomy, but suffered a relapse with platelet counts ranging from 25 to 108 x 10(9)/l over the next 3 years. However, following a severe Varicella infection 10 months ago, during which he developed a marked transient thrombocytosis, he has also maintained a normal platelet count.
authors.affiliation: Division of Haematology, United Medical and Dental Schools, St Thomas' Hospital, London.
authors.forename: J
authors.initials: J
authors.lastname: Beard
authors.forename: G F
authors.initials: GF
authors.lastname: Savidge
mesh_terms: D000293:Adolescent; D000328:Adult; D006679:HIV Seropositivity / Q000150:complications*; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D007116:Immunization, Passive; D007136:Immunoglobulins / Q000008:administration & dosage; D008297:Male; D013156:Splenectomy; D013601:T-Lymphocytes / Q000145:classification; D013921:Thrombocytopenia / Q000097:blood / Q000209:etiology / Q000628:therapy*
chemical_list: D007136:Immunoglobulins