同种异体干细胞移植后EBV相关的移植后淋巴增生性疾病并发噬细胞性淋巴组织细胞增多症:一种罕见的致命并发症

Tangia Muquith
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引用次数: 0

摘要

Epstein -Barr病毒相关的移植后淋巴细胞增生性疾病是一种致命和危及生命的并发症,因为免疫功能低下嗜血球淋巴组织细胞增多症是同种异体干细胞移植后EBV相关PTLD预后不良的标志。当体内T细胞耗竭策略被应用时,这是特别常见的在这两种情况下,移植后淋巴细胞增生性疾病和噬血细胞性淋巴组织细胞增多症,EBV感染是关键机制。在此,我报告一例29岁女性患者,在第二次异体干细胞移植后出现急性髓性白血病,并发PTLD并继发于eb病毒(EBV)感染的噬血细胞淋巴组织细胞增多症。患者接受了化学免疫疗法治疗,但最终在移植100天后死亡。HLH和EBV相关PTLD的关联是罕见的,这些患者的结果数据有限,死亡率非常高。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
EBV Associated Post-Transplant Lymphoproliferative Disorder Complicated with Haemophagocytic Lymphohistiocytosis After Allogeneic Stem Cell Transplant: A Rare Complication with Fatal Outcome
Epstein –Barr virus related post-transplant lymphoproliferative disorder is a fatal and life-threatening complication because of immunocompromised state.1 Haemophagocytic lymphohistiocytosis is a sign of poor outcome in EBV associated PTLD after allogeneic stem cell transplantation. It is particularly common when in vivo T cell depletion strategies have been applied.2 In both situations, post-transplant lymphoproliferative disorder and Haemophagocytic lymphohistiocytosis, infection with EBV is the key mechanism. Here I present a case of 29 years old female with acute myeloid leukaemia after second allogeneic stem cell transplant, who developed PTLD complicated with Haemophagocytic lymphohistiocytosis secondary to Epstein – Barr virus (EBV) infection. Patient was treated with chemo immunotherapy and responded but ultimately died after 100 days of transplant. The association of HLH and EBV related PTLD is rare and data on outcome of these patients are limited with very high mortality.
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