Department of Hepato-Pancreato-Biliary, Kyoto University Hospital, Kyoto 606-8507, Japan.
World J Gastroenterol. 2011 Apr 14;17(14):1848-57. doi: 10.3748/wjg.v17.i14.1848.
To investigate thrombotic microangiopathy (TMA) in liver transplantion, because TMA is an infrequent but life-threatening complication in the transplantation field.
A total of 206 patients who underwent living-donor liver transplantation (LDLT) were evaluated, and the TMA-like disorder (TMALD) occurred in seven recipients.
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.
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.
研究肝移植中的血栓性微血管病（TMA），因为 TMA 是移植领域中一种不常见但危及生命的并发症。
共评估了 206 例接受活体供肝移植（LDLT）的患者，其中 7 例受者发生 TMA 样疾病（TMALD）。
与其他 199 例受者相比，这些 TMALD 受者的预后较差。尽管有 2 例 TMALD 受者成功恢复，但其他 5 例受者最终死亡，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 后的肝活检组织病理学分析显示，根据结局存在明显差异。所有患者的抗去整合素样金属蛋白酶与血小板反应蛋白 1 型（ADAMTS-13）抗体的定性分析均为阴性。碎裂红细胞、微出血斑和血小板计数是 LDLT 后怀疑 TMALD 的早期标志物。尽管 vWF（von Willebrand 因子）和 ADAMTS-13 的绝对值不一定反映 TMALD，但 vWF/ADAMTS-13 比值在所有病例中均具有明确的诊断价值。必须根据每个病例决定是否进行 TMALD 的充分治疗，例如 PE 补充 ADAMTS-13 或治疗抑制性抗体。
通过可靠的标志物尽早识别 TMALD 并进行适当的治疗，可能为 LDLT 后的 TMALD 带来很大优势。
