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移植物抗宿主病或中毒性表皮坏死松解症:肝移植后的诊断困境

Graft-versus-host disease or toxic epidermal necrolysis: diagnostic dilemma after liver transplantation.

作者信息

Jeanmonod P, Hubbuch M, Grünhage F, Meiser A, Rass K, Schilling M K, Kollmar O

机构信息

Department of General, Visceral, Vascular and Pediatric Surgery, University of Saarland, Homburg-Saar, Germany.

出版信息

Transpl Infect Dis. 2012 Aug;14(4):422-6. doi: 10.1111/j.1399-3062.2012.00746.x. Epub 2012 Jun 1.

DOI:10.1111/j.1399-3062.2012.00746.x
PMID:22650490
Abstract

Graft-versus-host disease (GvHD) and toxic epidermal necrolysis (TEN) are rare and severe complications after liver transplantation. While mild acute GvHD is quite different from TEN and easy to distinguish, severe acute GvHD and TEN can be hard to differentiate because of similar clinical symptoms. We herein report a case with rapid progression of critical illness, after liver transplantation, caused by GvHD or TEN, although between those, diagnosis was not possible during the clinical course. Although, based on the timing/progression of the symptoms and the chimerism of >40%, the case seemed much more clinically consistent with GVHD, the combination of clinical symptoms together with skin rashes and the histologic appearance of skin lesions indicated diagnosis of a Stevens-Johnson syndrome/TEN overlap. The true diagnostic dilemma in such cases is discussed in detail, as these cases emphasize the need for more advanced diagnostic techniques.

摘要

移植物抗宿主病(GvHD)和中毒性表皮坏死松解症(TEN)是肝移植后罕见且严重的并发症。轻度急性GvHD与TEN有很大不同,易于区分,但严重急性GvHD和TEN由于临床症状相似,可能难以鉴别。我们在此报告1例肝移植后由GvHD或TEN导致的危重症快速进展病例,尽管在临床过程中无法对两者进行诊断。尽管根据症状出现的时间/进展以及嵌合率>40%,该病例在临床上似乎更符合GVHD,但临床症状与皮疹及皮肤病变的组织学表现相结合提示诊断为史蒂文斯-约翰逊综合征/TEN重叠。此类病例中真正的诊断困境将进行详细讨论,因为这些病例强调了对更先进诊断技术的需求。

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