| Title | Primary antiphospholipid syndrome with thrombotic thrombocytopenic purpura: a very unusual association. | | Author(s) | Carvalho J, Freitas C, Lima I, Leite C, Lage L | | Institution | Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo e Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo jotafc@gmail.com. | | Source | Lupus 2009; 18(9):841-4. | | 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. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 19578110 |
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