肝移植治疗朗格汉斯细胞组织细胞增多症后发生的严重硬化性胆管炎：1例成人病例报告
Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation: An adult case report.
作者信息
Tang Yunhua, Zhang Zhiheng, Chen Maogen, Ju Weiqiang, Wang Dongping, Ji Fei, Ren Qingqi, Guo Zhiyong, He Xiaoshun
机构信息
Organ Transplant Center, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
出版信息
Medicine (Baltimore). 2017 Mar;96(9):e5994. doi: 10.1097/MD.0000000000005994.
BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis. LCH diseases may occur in a single organ or multisystem organ. The patients with multisystem involvement usually have a poor prognosis. Liver involvement in multisystem LCH results in severe complications, such as obvious sclerosing cholangitis (SC) with jaundice.
METHODS
We reported a 31-year-old man developed severe SC due to multisystem LCH and was successfully treated by liver transplantation (LT). In addition, we firstly used tacrolimus and mycofenolate mofetil as immunosuppressants to treat LCH after LT.
RESULTS
We performed the immunosuppressants to deal with the LCH after LT, now the patient is currently well with normal liver function and no evidence of recurrence of LCH for 4 and a half years follow-up.
CONCLUSION
LT should be recommended as an effective treatment for these adults with severe SC due to multisystem LCH. Finally, using tacrolimus and mycofenolate mofetil as immunosuppressants to treat LCH might be favorable to prevent LCH recurrence.
背景
朗格汉斯细胞组织细胞增多症（LCH）是一种发病机制不明的罕见造血系统疾病。LCH疾病可发生于单个器官或多系统器官。多系统受累的患者通常预后较差。多系统LCH累及肝脏会导致严重并发症，如伴有黄疸的明显硬化性胆管炎（SC）。
方法
我们报告了一名31岁男性因多系统LCH发生严重SC，并通过肝移植（LT）成功治疗。此外，我们首次使用他克莫司和霉酚酸酯作为免疫抑制剂在LT后治疗LCH。
结果
我们在LT后使用免疫抑制剂处理LCH，目前患者情况良好，肝功能正常，在4年半的随访中无LCH复发迹象。
结论
对于这些因多系统LCH导致严重SC的成年人，应推荐LT作为一种有效的治疗方法。最后，使用他克莫司和霉酚酸酯作为免疫抑制剂治疗LCH可能有利于预防LCH复发。
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