| Title | Thrombotic microangiopathy in patients with phosphatidylserine dependent antiprothrombin antibodies and antiphospholipid syndrome. | | Author(s) | Kon Y, Atsumi T, Hagiwara H, Furusaki A, Kataoka H, Horita T, Yasuda S, Amengual O, Takao K | | Institution | Department of Medicine II, Hokkaido University Graduate School of Medicine, Sapporo, Japan. | | Source | Clin Exp Rheumatol 2008 Jan-Feb; 26(1):129-32. | | Abstract | Thrombotic microangiopathy (TMA) is a rare disorder characterized by microvascular thrombosis. TMA has been reported in patients with antiphospholipid antibodies and/or antiphospholipid syndrome but its pathogenesis is not clarified. We present two patients with TMA associated with IgG phosphatidylserine dependent antiprothrombin antibodies (aPS/PT). Case 1: A 44-year-old Japanese fem-ale with systemic lupus erythematosus (SLE) and positive lupus anticoagulant (LA) was started on ticlopidine after having stroke. Four weeks later she developed TMA. IgG/M/A anticardiolipin antibodies (aCL) were negative, but strong positive IgG aPS/PT were detected. Case 2: A 32-year-old Russian female with SLE was admitted because of hypertension, renal insufficiency and proteinuria at 14 weeks of pregnancy. She developed TMA after surgical abortion. IgG aPS/PT and LA were strongly positive but IgG/M/A aCL were negative. Neither case had von Willebrand factor cleaving protease (ADAMTS-13), suggesting that TMA in those patients was associated with thrombophilia rather than insufficient ADAMTS-13. Both patients were successfully treated with a series of plasma exchange. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 18328160 |
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