Kizaki M, Ikeda Y
Department of Internal Medicine, Keio University School of Medicine.
Nihon Rinsho. 1993 Jan;51(1):117-21.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disorder associated with multiple causes and occurring mainly in adults with multiorgan microvascular thrombosis. The high mortality of this disease needs rapid diagnosis and immediate therapy including plasmapheresis, plasma infusions, corticosteroids and immunosuppressive therapy, which have been improved survival rates of this disorder. Red cell fragmentation due to microangiopathic hemolytic anemia (MAHA) is one of the most characteristic findings in TTP. Identification of red cell fragments and hemolysis is, therefore, important for its rapid diagnosis. It is thought that hemolytic anemia is a consequence of mechanical damage to erythrocytes by fibrin strands, as they traverse peripheral microvessels. However, little is known of the mechanisms of red cell fragmentation and its relation to pathogenesis of TTP. We reviewed red cell deformity and its association to hemolysis by the points of physiologic, chemical and clinical aspects of erythrocytes.
血栓性血小板减少性紫癜（TTP）是一种罕见的、危及生命的疾病，病因多样，主要发生于患有多器官微血管血栓形成的成年人。该疾病的高死亡率需要快速诊断和立即治疗，包括血浆置换、血浆输注、皮质类固醇和免疫抑制治疗，这些措施提高了该疾病的生存率。微血管病性溶血性贫血（MAHA）导致的红细胞碎片是TTP最具特征性的表现之一。因此，识别红细胞碎片和溶血对其快速诊断很重要。据认为，溶血性贫血是纤维蛋白丝在穿过外周微血管时对红细胞造成机械损伤的结果。然而，关于红细胞碎片的机制及其与TTP发病机制的关系知之甚少。我们从红细胞的生理、化学和临床方面回顾了红细胞畸形及其与溶血的关系。
