异基因造血干细胞移植后EB病毒相关淋巴增殖性疾病临床病程的异质性。
Heterogeneity in clinical course of EBV-associated lymphoproliferative disorder after allogeneic stem cell transplantation.
作者信息
Meyer Sara C, Medinger Michael, Halter Jörg P, Baldomero Helen, Hirsch Hans H, Tzankov Alexandar, Dirnhofer Stephan, Passweg Jakob R, Tichelli André
出版信息
Hematology. 2014 Jul;19(5):280-5. doi: 10.1179/1607845413Y.0000000123. Epub 2013 Nov 25.
OBJECTIVE AND IMPORTANCE
Post-transplant lymphoproliferative disorder (PTLD) is a severe complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT) associated with Epstein-Barr virus (EBV).
CLINICAL PRESENTATIONS
Among 263 individuals treated with allo-HSCT for severe aplastic anemia, pure white cell aplasia, T-prolymphocytic leukemia, and relapsed Hodgkin lymphoma, we diagnosed EBV-PTLD in 5 patients. Median age was 29 years (range 19-70 years) and four of five patients were EBV-seropositive prior to HSCT. All five had unrelated EBV-positive donors. In all cases, PTLD occurred within the first year post-transplant (median 4 months).
INTERVENTION
There were two rapidly fatal courses with extensive organ involvement. Both patients showed lymphopenia and thrombocytopenia. In contrast, the three surviving patients had higher lymphocytes and normal platelet counts, while PTLD was restricted to one site and resolved after 2-4 cycles of rituximab.
CONCLUSION
In this case series courses of PTLD showed substantial diversity.
目的及重要性
移植后淋巴细胞增殖性疾病（PTLD）是异基因造血干细胞移植（allo-HSCT）后与 Epstein-Barr 病毒（EBV）相关的一种严重并发症。
临床表现
在 263 例因严重再生障碍性贫血、纯白细胞再生障碍、T 前淋巴细胞白血病及复发性霍奇金淋巴瘤接受 allo-HSCT 治疗的个体中，我们诊断出 5 例 EBV-PTLD。中位年龄为 29 岁（范围 19 - 70 岁），5 例患者中有 4 例在 HSCT 前 EBV 血清学呈阳性。所有 5 例均有不相关的 EBV 阳性供者。所有病例中，PTLD 均发生在移植后第一年（中位时间 4 个月）。
干预措施
有 2 例病程迅速进展且广泛累及器官，均迅速死亡。这 2 例患者均表现为淋巴细胞减少和血小板减少。相比之下，3 例存活患者淋巴细胞计数较高且血小板计数正常，PTLD 局限于一个部位，在接受 2 - 4 个周期利妥昔单抗治疗后缓解。
结论
在该病例系列中，PTLD 的病程表现出显著差异。
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