title: Primary antiphospholipid syndrome with thrombotic thrombocytopenic purpura: a very unusual association.
abstract: This report considers the rare situation in which primary antiphospholipid syndrome (PAPS) is linked with thrombotic thrombocytopenic purpura (TTP). It describes the case of a young lady with PAPS, characterized by recurring cerebro-vascular abnormalities and marked livedo reticularis, combined with circulating anticardiolipin and lupus anticoagulant antibodies. On follow-up, while on oral anticoagulation, she developed severe thrombocytopenia associated with hematuria, microangiophatic anaemia and neurological manifestations consistent with a diagnosis of TTP. The patient was treated with pulses of methylprednisolone and plasmapheresis with plasma exchange. The result was a favourable outcome. To our knowledge, this is the seventh report on this rare association in the English-language literature of this field.
authors: Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo e Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo-SP, Brazil. jotafc@gmail.com
authors: Jozélio Freire
authors: JF
authors: de Carvalho
authors: C A E W
authors: CA
authors: Freitas
authors: I V S
authors: IV
authors: Lima
authors: C C
authors: CC
authors: Leite
authors: L V
authors: LV
authors: Lage
mesh_terms: D000328:Adult; D000906:Antibodies / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D002308:Cardiolipins / Q000276:immunology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000276:immunology; D008775:Methylprednisolone / Q000627:therapeutic use; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D000906:Antibodies; D002308:Cardiolipins; D005938:Glucocorticoids; D016682:Lupus Coagulation Inhibitor; D008775:Methylprednisolone