The initial evaluation of a patient with suspected thrombotic thrombocytopenic purpura (TTP) or another primary thrombotic microangiopathy (TMA) syndrome must focus on distinguishing these primary syndromes from other systemic disorders that can present with microangiopathic hemolytic anemia (MAHA) and thrombocytopenia.

Mar 24, 2025 · TTP can be immune-mediated, due to autoantibodies against ADAMTS13, or hereditary, due to pathogenic variants in the ADAMTS13 gene. This topic reviews the clinical manifestations and our approach to the diagnosis of immune TTP.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13 (usually <10 percent).

Nov 29, 2022 · Thrombotic microangiopathy (TMA) describes a specific pathologic lesion in which abnormalities in the vessel wall of arterioles and capillaries lead to microvascular thrombosis.

对于疑似血栓性血小板减少性紫癜 (thrombotic thrombocytopenic purpura, TTP)或其他原发性血栓性微血管病 (thrombotic microangiopathy, TMA)综合征的患者，初始评估必须侧重于将这些原发性综合征与其他可表现为微血管病性溶血性贫血 (microangiopathic hemolytic anemia, MAHA)和血小 …

This differs from thrombotic thrombocytopenic purpura (TTP), which can be diagnosed using an ADAMTS13 assay and is rarely associated with AKI. This topic discusses our approach to the evaluation and management of ST-HUS and CM-TMA in adults.

Drug-induced TMA (DITMA) is challenging to diagnose because laboratory tests to identify a drug etiology may not be available. Some of the implicated substances may not be acknowledged by the patient, and quinine exposure may be from a beverage such as tonic water.

本文将总结以下疾病的病理生理：免疫性TTP、补体介导性TMA (complement-mediated TMA, CM-TMA)、志贺毒素 (Shiga toxin, ST)诱发性TMA [ST介导溶血尿毒综合征 (shiga toxin-mediated hemolytic-uremic syndrome, ST-HUS)]及一些罕见的遗传性TMA。

与很少引起AKI的血栓性血小板减少性紫癜 (thrombotic thrombocytopenic purpura, TTP)相比，主要表现为AKI的成人血栓性微血管病 (thrombotic microangiopathy, TMA)综合征更不易识别且相关报道更少，如志贺毒素介导 (腹泻)的溶血尿毒综合征 (Shiga toxin-mediated hemolytic uremic syndrome, ST-HUS ...

Jan 15, 2025 · The initial evaluation of a patient with suspected thrombotic thrombocytopenic purpura (TTP) or another primary thrombotic microangiopathy (TMA) syndrome must focus on distinguishing these primary syndromes from other systemic disorders that can present with microangiopathic hemolytic anemia (MAHA) and thrombocytopenia.