TY - JOUR
T1 - Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation
AU - Tang, Yunhua
AU - Zhang, Zhiheng
AU - Chen, Maogen
AU - Ju, Weiqiang
AU - Wang, Dongping
AU - Ji, Fei
AU - Ren, Qingqi
AU - Guo, Zhiyong
AU - He, Xiaoshun
N1 - Publisher Copyright:
Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc.
Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 2017/3/1
Y1 - 2017/3/1
N2 - 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.
AB - 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.
KW - Langerhans cell histiocytosis
KW - liver transplantation
KW - sclerosing cholangitis
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U2 - 10.1097/MD.0000000000005994
DO - 10.1097/MD.0000000000005994
M3 - Article
C2 - 28248858
AN - SCOPUS:85015898427
VL - 96
JO - Medicine (United States)
JF - Medicine (United States)
SN - 0025-7974
IS - 9
M1 - e5994
ER -