- TTP(thrombotic thrombocytopenic purpora)
- HUS(hemolytic uremic syndrome)
- HSP(Henoch-Schönlein purpura)
혈전미세혈관병증(thrombotic microangiopathy, TMA)이란?
- 병리학적으로 미세순환에서 적혈구 파괴에 의한 미세혈관병용혈빈혈(microangiopathic hemolytic anemia), 혈전에 의한 전반적인 미세혈관폐쇄와 혈소판파괴에 의한 혈소판감소증을 보이는 질환군(thrombotic micrangiopathy; a pathology that results in thrombosis in capillaries and arterioles, due to an endothelial injury characterized by hemolytic anemia and thrombocytopenia)
혈전미세혈관병증의 분류
선천혈전혈소판감소자색반병 | |
특발혈전혈소판감소자색반병 | - 후천 ADAMTS13 결핍형
- 후천 ADAMTS13 무결핍형
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이차혈전미세혈관병증 | - 감염/DIC
- 조혈이식 관련
- 암
- 임신 관련: 전자간증, 자간증, HELLP 증후군
- 자가면역질환: EVANS 증후군, 전신홍반루푸스, 항인지질증후군
- 약물
- 기타: 수술 후, 악성고혈압
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용혈요독증후군 | |
병태생리
- Microvascular thrombosis
- Thrombocytopenia
- Microangiopathic hemolytic anemia
- EHEC: Enterohemorrhagic E. coli
- Stx1/Stx2: Shiga-like toxins 1/2
- UL-VWF: ultra-large VWF → prothrombotic하다.
- CFI: complement factor I; CFH: complement factor H; CFB: complement factor B
- https://docs.google.com/presentation/d/1B8TPNajJrb9alH6ItXOj7LTk4ciqW67jHy3YCbedNtg/edit#slide=id.g1c10e0ac45_0_488
혈전미세혈관병증의 감별진단
ADAMTS13=a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13
aHUS=atypical hemolytic uremic syndrome; eGFR=estimated glomerular filtration rate; EHEC=Escherichia coli; LDH=lactate dehydrogenase; STEC-HUS=Shiga toxin–producing Escherichia coli (STEC) hemolytic uremic syndrome; TTP=thrombotic thrombocytopenic purpura
참고자료
- George JN, Nester CM. N Engl J Med 2014;371:654-66
- Moake JL. N Engl Med M 2002;347:589-600
- 대한혈액학회. 혈액학 3판(2018). 47.4장
- Mayer CL, Leibowitz CS, Kurosawa S, Stearns-Kurosawa DJ. Shiga toxins and the pathophysiology of hemolytic uremic syndrome in humans and animals. Toxins (Basel) 2012;4(11):1261–87.
- Laurence J. Atypical hemolytic uremic syndrome (aHUS): making the diagnosis. Clin Adv Hematol Oncol 2012;10(10 Suppl 17):1–12.