Stréhn Anita, Szőnyi László, Kriván Gergely, Kovács Lajos, Reusz György, Szabó Attila, Rényi Imre, Kovács Gábor, Dezsőfi Antal
Egyesített Szt. István és Szt. László Kórház Gyermekhematológiai és Őssejt-transzplantációs Osztály Budapest.
Semmelweis Egyetem, Általános Orvostudományi Kar I. Gyermekgyógyászati Klinika Budapest Bókay J. u. 53. 1083.
Orv Hetil. 2014 Feb 23;155(8):313-8. doi: 10.1556/OH.2014.29796.
Among possible complications of transplantation the post-transplant lymphoproliferative disease due to immunosuppressive therapy is of paramount importance. In most cases the direct modulating effect of Epstein-Barr virus on immune cells can be documented.
The aim of the authors was to evaluate the incidence os post-transplant lymphoproliferative diseases in pediatric transplant patients in Hungary.
The study group included kidney, liver and lung transplant children followed up at the 1st Department of Pediatrics, Semmelweis University, Budapest and stem cell transplant children at Szent László Hospital, Budapest. Data were collected from 78 kidney, 109 liver and 17 lung transplant children as well as from 243 children who underwent allogenic stem cell transplantation.
Between 1998 and 2012, 13 children developed post-transplant lymphoproliferative disorder (8 solid organ transplanted and 5 stem cell transplanted children). The diagnosis was based on histological findings in all cases. Mortality was 3 out of the 8 solid organ transplant children and 4 out of the 5 stem cell transplant children. The highest incidence was observed among lung transplant children (17.6%).
These data indicate that post-transplant lymphoproliferative disease is a rare but devastating complication of transplantation in children. The most important therapeutic approaches are reduction of immunosuppressive therapy, chemotherapy and rituximab. Early diagnosis may improve clinical outcome and, therefore, routine polymerase chain reaction screening for Epstein-Barr virus of high risk patients is recommended.
在移植的可能并发症中，免疫抑制治疗导致的移植后淋巴细胞增生性疾病至关重要。在大多数情况下，可证明爱泼斯坦-巴尔病毒对免疫细胞有直接调节作用。
作者的目的是评估匈牙利儿科移植患者中移植后淋巴细胞增生性疾病的发病率。
研究组包括在布达佩斯塞梅尔维斯大学第一儿科随访的肾、肝和肺移植儿童，以及布达佩斯圣拉兹洛医院的干细胞移植儿童。收集了78例肾移植儿童、109例肝移植儿童、17例肺移植儿童以及243例接受同种异体干细胞移植儿童的数据。
1998年至2012年期间，13名儿童发生了移植后淋巴细胞增生性疾病（8名实体器官移植儿童和5名干细胞移植儿童）。所有病例的诊断均基于组织学检查结果。8名实体器官移植儿童中有3例死亡，5名干细胞移植儿童中有4例死亡。肺移植儿童中的发病率最高（17.6%）。
这些数据表明，移植后淋巴细胞增生性疾病是儿童移植中一种罕见但具有破坏性的并发症。最重要的治疗方法是减少免疫抑制治疗、化疗和利妥昔单抗。早期诊断可能改善临床结局，因此，建议对高危患者进行常规的爱泼斯坦-巴尔病毒聚合酶链反应筛查。
