Thrombotic microangiopathy-like disorder after living-donor liver transplantation: a single-center experience in Japan.
Thrombotic microangiopathy-like disorder after living-donor liver transplantation: a single-center experience in Japan.
复制标题
活体肝移植后血栓性微血管病样疾病：日本的单中心经验。
DOI：
10.3748/wjg.v17.i14.1848
复制
发表时间：
2011
影响因子：
4.3
通讯作者：
S. Uemoto
中科院分区：
文献类型：
--
作者：
T. Hori;T. Kaido;F. Oike;Y. Ogura;K. Ogawa;Y. Yonekawa;K. Hata;Y. Kawaguchi;M. Ueda;A. Mori;H. Segawa;K. Yurugi;Y. Takada;H. Egawa;A. Yoshizawa;Takuma Kato;Kanako Saito;Linan N. Wang;M. Torii;Feng Chen;Ann;Lindsay B. Gardner;S. Uemoto
目的
探讨血栓性微血管病（TMA）在肝移植中的作用，因为TMA是移植领域中罕见但危及生命的并发症。
方法
共有206例接受活体肝移植（LDLT）的患者进行了评估，并在7名受者中发生了TMA样疾病（TMALD）。
结果
与其他199名接受者相比，这些TMALD接受者的结果较差。尽管两名TMALD受体成功康复，但其他五名受体最终死亡，尽管进行了包括重复血浆置换（PE）和再次移植在内的强化治疗。LDLT后肝活检的组织学分析显示，根据结局有明显差异。定性分析抗体对去整合素样结构域和金属蛋白酶与血小板反应蛋白1型基序（ADAMTS-13）是阴性的所有患者。红细胞碎裂、微出血斑和血小板计数是LDLT后怀疑TMALD的早期指标。虽然血管性血友病因子（vWF）和ADAMTS-13的绝对值并不一定反映TMALD，但vWF/ADAMTS-13比值在所有病例中都具有明确的诊断价值。必须根据每种情况确定TMALD的适当治疗方法，例如ADAMTS-13补充的PE或抑制性抗体治疗。
结论
根据可靠的标志物早期识别TMALD，最佳诱导适当的治疗可能为LDLT后的TMALD提供很大的优势。
AIM
To investigate thrombotic microangiopathy (TMA) in liver transplantion, because TMA is an infrequent but life-threatening complication in the transplantation field.
METHODS
A total of 206 patients who underwent living-donor liver transplantation (LDLT) were evaluated, and the TMA-like disorder (TMALD) occurred in seven recipients.
RESULTS
These TMALD recipients showed poor outcomes in comparison with other 199 recipients. Although two TMALD recipients successfully recovered, the other five recipients finally died despite intensive treatments including repeated plasma exchange (PE) and re-transplantation. Histopathological analysis of liver biopsies after LDLT revealed obvious differences according to the outcomes. Qualitative analysis of antibodies against a disintegrin-like domain and metalloproteinase with thrombospondin type 1 motifs (ADAMTS-13) were negative in all patients. The fragmentation of red cells, the microhemorrhagic macules and the platelet counts were early markers for the suspicion of TMALD after LDLT. Although the absolute values of von Willebrand factor (vWF) and ADAMTS-13 did not necessarily reflect TMALD, the vWF/ADAMTS-13 ratio had a clear diagnostic value in all cases. The establishment of adequate treatments for TMALD, such as PE for ADAMTS-13 replenishment or treatments against inhibitory antibodies, must be decided according to each case.
CONCLUSION
The optimal induction of adequate therapies based on early recognition of TMALD by the reliable markers may confer a large advantage for TMALD after LDLT.
丙型肝炎阳性同种异体肾移植受者中与抗心磷脂抗体相关的肾血栓性微血管病。
DOI：
10.1681/asn.v101146
发表时间：
1999
期刊：
Journal of the American Society of Nephrology : JASN
影响因子：
--
作者：
Baid,S;Pascual,M;WilliamsJr,WW;Tolkoff-Rubin,N;Johnson,SM;Collins,B;Chung,RT;Delmonico,FL;Cosimi,AB;Colvin,RB
通讯作者：
Colvin,RB
影响因子：
158.5
作者：
BELL, WR;BRAINE, HG;KICKLER, TS
通讯作者：
KICKLER, TS