Itoh Y, Sekine H, Hosono O, Takeuchi T, Koide J, Takano M, Abe T
The 2nd Department of Internal Medicine, Saitama Medical Center, Japan.
Clin Immunol Immunopathol. 1990 Oct;57(1):125-36. doi: 10.1016/0090-1229(90)90028-o.
Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome of unknown etiology and has a high mortality rate due to disseminated platelet thrombi. However, the mechanism of platelet agglutination is not understood. Although an immunological mechanism has been suggested as the basis for the pathogenesis of TTP, any possible immune-mediated etiology remains unclear. The association of TTP with systemic lupus erythematosus (SLE) affords a unique opportunity to study such possibilities, because SLE is a prototype of autoimmune disease. This report describes two patients with SLE who developed TTP. The development of anti-platelet antibodies is one possible immunological mechanism for platelet agglutination in patients with SLE complicated by TTP. More importantly, patient J.Y., who had anti-platelet antibodies, responded dramatically to high doses of prednisolone.
血栓性血小板减少性紫癜（TTP）是一种病因不明的临床综合征，由于弥散性血小板血栓形成，其死亡率很高。然而，血小板凝集的机制尚不清楚。虽然有免疫机制被认为是TTP发病机制的基础，但任何可能的免疫介导病因仍不明确。TTP与系统性红斑狼疮（SLE）的关联为研究这些可能性提供了独特的机会，因为SLE是自身免疫性疾病的典型代表。本报告描述了两名患有SLE并发展为TTP的患者。抗血小板抗体的产生是SLE合并TTP患者血小板凝集的一种可能免疫机制。更重要的是，患有抗血小板抗体的J.Y.患者对大剂量泼尼松龙反应显著。
