title: Thrombotic thrombocytopaenic purpura in HIV-infected patients.
abstract: Thrombotic thrombocytopaenic purpura (TTP) results from deficiency of von Willebrand factor-cleaving protease (vWF-cp) activity. Eight HIV-infected patients presented with TTP, representing 12.5% of all TTP treated at this centre. In four patients presentation with TTP revealed underlying HIV infection, the other four patients were previously known to be HIV infected, with plasma exchange and highly active antiretroviral therapy (HAART) all recovered. Normalization of vWF-cp activity was associated with recovery. Relapse occurred in two patients who discontinued HAART against medical advice, suggesting that HIV has a causal role in this condition. Given the clear benefit from HAART in addition to plasma exchange, these data suggest that all patients presenting with TTP should undergo HIV testing.
authors.affiliation: Centre for Sexual Health and HIV Research, Department of Primary Care and Population Sciences, Royal Free and University College Medical School, University College London, Mortimer Market Centre, Camden PCT, London WC1E 6AU, UK.
authors.forename: R F
authors.initials: RF
authors.lastname: Miller
authors.forename: M
authors.initials: M
authors.lastname: Scully
authors.forename: H
authors.initials: H
authors.lastname: Cohen
authors.forename: S
authors.initials: S
authors.lastname: Roedling
authors.forename: R
authors.initials: R
authors.lastname: Starke
authors.forename: S G
authors.initials: SG
authors.lastname: Edwards
authors.forename: S J
authors.initials: SJ
authors.lastname: Machin
mesh_terms: D023241:Antiretroviral Therapy, Highly Active; D006678:HIV; D015658:HIV Infections / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008666:Metalloendopeptidases / Q000172:deficiency*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000209:etiology*; D012008:Recurrence; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D014841:von Willebrand Factor; D008666:Metalloendopeptidases