title: Inhibitor development and successful immune tolerance in an HIV-infected patient with haemophilia A and after immune reconstitution with HAART.
abstract: This is the case of a 28-year-old man with severe congenital haemophilia A, who had a relatively mild bleeding course during early childhood, with limited factor VIII (FVIII) exposure. He was infected with HIV before the age of 7 years, and demonstrated profound immunodeficiency from childhood, with very low CD4+ cell counts for more than a decade. Following initiation of highly active anti-retroviral therapy (HAART) and gradually increasing CD4+ cells, he presented for the first time with a high-titre inhibitor at age 26, despite over 1000 previous FVIII exposures. Subsequently, his inhibitor was successfully eradicated with a standard immune tolerance protocol. It is likely that the effects of chronic HIV infection on T-lymphocyte pathways, and the partial immune reconstitution resulting from HAART, led to this patient's unusual inhibitor course. Such a case serves to augment knowledge gained in animal studies about the immunobiology of FVIII inhibitors.
authors.affiliation: Department of Medicine, Tulane University School of Medicine, New Orleans, LA 70112, USA. rkrusej@cox.net
authors.forename: R
authors.initials: R
authors.lastname: Kruse-Jarres
authors.forename: C M
authors.initials: CM
authors.lastname: Hadi
authors.forename: C A
authors.initials: CA
authors.lastname: Leissinger
mesh_terms: D000328:Adult; D023241:Antiretroviral Therapy, Highly Active / Q000379:methods; D019774:Blood Coagulation Factor Inhibitors / Q000276:immunology*; D018791:CD4 Lymphocyte Count; D015496:CD4-Positive T-Lymphocytes; D005169:Factor VIII / Q000276:immunology / Q000627:therapeutic use*; D015658:HIV Infections / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000276:immunology*; D007108:Immune Tolerance / Q000187:drug effects*; D008297:Male
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D005169:Factor VIII