Cobalamin Deficient Thrombotic Microangiopathy: a case of TTP or Pseudo-TTP

A. Franks, A. Jarrell, A. Mays, Tammy Bannister
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引用次数: 3

Abstract

A thrombotic microangiopathy (TMA) occurs when a patient presents with microangiopathic hemolytic anemia, thrombocytopenia and organ damage. There are many causes of TMAs, but thrombotic thrombocytopenic purpura (TTP) must always be considered because of its high mortality rate. Treatment with therapeutic plasma exchange and transfusions can reduce the mortality rate but those treatments carry their own morbidity. Severe cobalamin deficiency can cause a clinical picture similar to TTP. Understanding the pathologic changes of this pseudo-TTP can allow physicians to suspect this difference early in the clinical course. In the following case, elevations in homocysteine and methylmalonic acid, as well as changes in the CBC, allowed this distinction to be identified early, minimizing potentially dangerous treatments.
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钴胺素缺乏性血栓性微血管病:TTP或伪TTP 1例
血栓性微血管病(TMA)发生时,患者表现为微血管致病性溶血性贫血,血小板减少和器官损害。TMAs有许多原因,但血栓性血小板减少性紫癜(TTP)必须考虑,因为它的高死亡率。治疗性血浆交换和输血可以降低死亡率,但这些治疗也有其自身的发病率。严重的钴胺素缺乏可引起类似TTP的临床症状。了解这种假性ttp的病理变化可以使医生在临床过程中早期怀疑这种差异。在以下病例中,同型半胱氨酸和甲基丙二酸的升高,以及CBC的变化,使这种区别能够及早发现,将潜在的危险治疗降到最低。
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