Newell K A, Alonso E M, Kelly S M, Rubin C M, Thistlethwaite J R, Whitington P F
Department of Surgery, University of Chicago, Illinois 60637, USA.
J Pediatr. 1997 Jul;131(1 Pt 1):98-104. doi: 10.1016/s0022-3476(97)70131-8.
Langerhans cell histiocytosis (LCH) is an unusual indication for orthotopic liver transplantation in children. Data from limited case reports suggest that orthotopic liver transplantation for LCH is associated with excellent survival rates and a low incidence of disease recurrence. However, in our experience, children who have transplantation for LCH appeared to experience a high incidence of refractory rejection and posttransplant lymphoproliferative disease (PTLD).
Data from 398 liver transplants performed in 298 children younger than 16 years of age were reviewed to determine the presence of risk factors for PTLD in patients with LCH and other causes of liver failure.
The incidence of PTLD was significantly higher in children who received transplants for LCH compared with all indications (p < 0.001) and specific indications that were associated with the development of PTLD (p < 0.002). Among patients in whom PTLD developed, there was no significant difference in the incidence of primary Epstein-Barr virus infections in patients who receive transplantation for LCH (4/4, 100%) versus all other indications (12/14, 86%). Children who had transplantation for LCH were older than those who had transplantation for other indications (LCH median age 3.1 years, other indications 1 year). The incidence of rejection, especially refractory rejection, was greater in patients who had transplantation for LCH (100% and 50%, respectively) compared with those who had transplantation for other indications (70% and 10%, p < 0.02 for refractory rejection).
Patients who had transplantation for liver disease related to LCH experienced a 67% long-term survival (median follow up 5.8 years, range 2.1 to 7.5 years). Recurrent LCH occurred in only 33% of patients and was easily managed. However, PTLD developed in two thirds of these patients, perhaps in part because of the high incidence of refractory rejection. This series therefore demonstrates an association between a primary disease process and the development of PTLD. Although the data indicate that children with LCH-induced liver failure benefit from transplantation, special care must be exercised in screening for and preemptive treatment of PTLD.
朗格汉斯细胞组织细胞增多症（LCH）是儿童原位肝移植的一种罕见适应证。有限病例报告的数据表明，LCH原位肝移植的生存率很高，疾病复发率很低。然而，根据我们的经验，因LCH接受移植的儿童似乎发生难治性排斥反应和移植后淋巴细胞增生性疾病（PTLD）的发生率很高。
回顾了298例16岁以下儿童进行的398例肝移植数据，以确定LCH患者和其他肝衰竭病因患者发生PTLD的危险因素。
与所有适应证（p < 0.001）以及与PTLD发生相关的特定适应证相比，因LCH接受移植的儿童PTLD发生率显著更高（p < 0.002）。在发生PTLD的患者中，因LCH接受移植的患者（4/4，100%）与所有其他适应证患者（12/14，86%）的原发性EB病毒感染发生率无显著差异。因LCH接受移植的儿童比因其他适应证接受移植的儿童年龄更大（LCH中位年龄3.1岁，其他适应证为1岁）。与因其他适应证接受移植的患者相比，因LCH接受移植的患者排斥反应发生率更高，尤其是难治性排斥反应（分别为100%和50%，难治性排斥反应p < 0.02）（其他适应证为70%和10%）。
因与LCH相关的肝病接受移植的患者长期生存率为67%（中位随访5.8年，范围2.1至7.5年）。仅33%的患者发生复发性LCH，且易于处理。然而，这些患者中有三分之二发生了PTLD，可能部分原因是难治性排斥反应发生率高。因此，本系列研究证明了原发性疾病过程与PTLD发生之间的关联。虽然数据表明LCH所致肝衰竭的儿童可从移植中获益，但在筛查和预防性治疗PTLD时必须格外小心。
