Ekman-Joelsson Britt-Marie, Wåhlander Håkan, Synnergren Mats, Sager Madeleine, Mellgren Karin
1Department of Pediatrics,Institution for Clinical Sciences,Sahlgrenska Academy,University of Gothenburg,Gothenburg,Sweden.
2Södra Älvsborg Hospital,Borås,Sweden.
Cardiol Young. 2017 Nov;27(9):1823-1831. doi: 10.1017/S104795111700155X. Epub 2017 Aug 7.
Heart transplantation has been an option for children in Sweden since 1989. As our unit faced an increased rate of post-transplant lymphoproliferative disorder, the objective of the study was to identify possible risk factors.
This is a retrospective study of all children aged 0-18 years who underwent heart transplantation in Gothenburg from 1989 to 2014.
A total of 71 children underwent heart transplantation. The overall incidence of post-transplant lymphoproliferative disorder was 14% (10/71); however, 17% (6/36) of those undergoing transplantation after 2007 developed lymphoma, compared with only 10% (4/35) of transplantation cases before 2007 (p=0.85). The mean age at transplantation was 9 years (0-17). The mean post-transplant follow-up time was 5.5 years (0.5-21.9) in the group that developed post-transplant lymphoproliferative disorder, compared with 10.2 years (0.02-25.2) in those who did not. In our study group, risk factors for post-transplant lymphoproliferative disorder were surgically palliated CHD (p=0.0005), sternotomy during infancy (p⩽0.0001), hypoplastic left ventricle (p=0.0001), number of surgical events (p=0.0022), mismatch concerning Epstein-Barr virus infection - that is, a positive donor-negative recipient (p⩽0.0001) - and immunosuppressive treatment with tacrolimus compared with ciclosporine (p=0.028). Discussion This study has three major findings. First, post-transplant lymphoproliferative disorder only developed in subjects born with CHD. Second, the vast majority (9/10) of the subjects developing the disorder had undergone sternotomy as infants. Third, the number of surgical events correlated with a higher risk for developing post-transplant lymphoproliferative disorder.
自1989年以来，心脏移植一直是瑞典儿童的一种治疗选择。由于我们科室面临移植后淋巴细胞增生性疾病发病率上升的问题，本研究的目的是确定可能的风险因素。
这是一项对1989年至2014年在哥德堡接受心脏移植的所有0至18岁儿童的回顾性研究。
共有71名儿童接受了心脏移植。移植后淋巴细胞增生性疾病的总体发病率为14%（10/71）；然而，2007年后接受移植的患者中有17%（6/36）发生淋巴瘤，而2007年前移植病例中只有10%（4/35）发生淋巴瘤（p=0.85）。移植时的平均年龄为9岁（0至17岁）。发生移植后淋巴细胞增生性疾病的组移植后的平均随访时间为5.5年（0.5至21.9年），未发生该疾病的组为10.2年（0.02至25.2年）。在我们的研究组中，移植后淋巴细胞增生性疾病的风险因素包括外科姑息性先天性心脏病（p=0.0005）、婴儿期胸骨切开术（p≤0.0001）、左心室发育不全（p=0.0001）、手术次数（p=0.0022）、与爱泼斯坦-巴尔病毒感染不匹配，即供体阳性受体阴性（p≤0.0001）以及与环孢素相比使用他克莫司进行免疫抑制治疗（p=0.028）。讨论本研究有三个主要发现。第一，移植后淋巴细胞增生性疾病仅在患有先天性心脏病的患者中发生。第二，绝大多数（9/10）发生该疾病的患者在婴儿期接受过胸骨切开术。第三，手术次数与发生移植后淋巴细胞增生性疾病的较高风险相关。
