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혈전미세혈관병증의 감별 진단과 치료

Differential Diagnosis and Treatment of Thrombotic Microangiopathy Syndrome

대한내과학회지 2019년 94권 1호 p.83 ~ 88
홍준식 ( Hong Jun-Shik ) - 서울대학교 의과대학 내과학교실

Abstract


Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.

키워드

Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura; Hemolytic uremic syndrome; Plasma exchange; Eculizumab
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