혈전미세혈관병증의 감별 진단과 치료

• Published in: The Korean journal of medicine Vol. 94; no. 1; pp. 83 - 88
• Main Authors: 홍준식, Junshik Hong
• Format: Journal Article
• Language: Korean
• Published: 대한내과학회 01.02.2019
• Subjects: Eculizumab; Hemolytic uremic syndrome; Plasma exchange; Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura; 내과학; 에쿨리주맙; 용혈요독증후군; 혈장교환술; 혈전미세혈관병증; 혈전성혈소판감소자반증
• ISSN: 1738-9364; 2289-0769
• DOI: 10.3904/kjm.2019.94.1.83

TMA는 다양한 원인에 의하여 발생하므로 감별 진단이 중요하며 신속한 초기 대처와 함께 전문적인 검사 결과들을 종합하여 확진해야 하는 질환이다. 최근 진단 검사법과 치료제의 발전이 두드러지는 질환군으로 정확한 진단과 그에 따른 올바른 치료법의 설정으로 치료 성적을 향상시킬 수 있다. 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. (Korean J Med 2019;94:83-88)