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.

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 …

Thrombotic microangiopathies (TMAs) are potentially life-threatening conditions caused by small-vessel platelet thrombi. Characteristic clinical features are microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. Acute kidney injury (AKI), neurologic abnormalities, and/or cardiac ischemia may be seen. (See 'Clinical manifestations' below.)

TMA: thrombotic microangiopathy; MAHA: microangiopathic hemolytic anemia; DIC: disseminated intravascular coagulation; HELLP: hemolysis, elevated liver function tests, low platelets; TTP: thrombotic thrombocytopenic purpura; HUS: hemolytic-uremic syndrome.

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.

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.

免疫性血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura, TTP)是原发性TMA中最早得到阐释的疾病，通过免疫性TTP也许最能了解TMA的病理生理学。在原发性TMA中，TTP是比较独特的，因为该病中微血栓虽然遍布肾脏，但肾功能异常却极轻微。

Jun 24, 2024 · It is characterized by small-vessel platelet-rich thrombi, thrombocytopenia, and microangiopathic hemolytic anemia (MAHA). Some patients may have neurologic abnormalities, mildly reduced kidney function, or low-grade fever. Immune TTP is …

其特征为小血管内产生富血小板血栓，引起血小板减少、微血管病性溶血性贫血(microangiopathic hemolytic anemia, MAHA)，有时还会引起器官损伤。TTP必须立刻治疗，否则死亡率接近100%。经适当治疗，预计生存率可超过90%。

Thrombotic thrombocytopenic purpura (TTP) May have severe neurologic abnormalities. Inherited; may present in a newborn infant, a child with thrombocytopenia, or, less commonly, an adult. Among adults, a common presentation is during a first pregnancy. Acquired autoimmune: Uncommon in children.