单倍体造血干细胞联合肝移植在儿科患者中的应用

Vedat Uygun, İbrahim Aliosmanoğlu, Hayriye Daloğlu, Seda Öztürkmen, Koray Yalçın, Gülsün Karasu, Akif Yeşilipek
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 A juvenile myelomonocytic leukemia patient, who underwent unmanipulated haplo-HSCT with PTCY from her mother at the age of 3 years, developed chronic liver graft versus host disease (GvHD) which was refractory to specific GvHD treatment. Liver transplantation (LT) from her mother (the donor of her haplo-HSCT) was decided as the next line of treatment.
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引用次数: 0

摘要

来自同一供体的实体器官移植是先前造血干细胞移植(HSCT)后发生的终末期器官衰竭的既定程序;然而,在未经操作的单倍造血干细胞移植患者中很少这样做。目前还没有关于单倍造血干细胞移植后使用环磷酰胺(PTCY)后器官移植的长期表现的儿科报告。一名少年骨髓单核细胞白血病患者在3岁时接受了未经处理的单倍造血干细胞移植,并从母亲那里获得了PTCY,患上了慢性肝移植物抗宿主病(GvHD),特异性GvHD治疗难治性。她的母亲(她的单倍hsct的供体)的肝移植(LT)被决定作为下一步的治疗方案。移植于造血干细胞移植后第540天进行,适当切除供体左外侧段并附着于受体。GvHD的症状在一个月内完全消退。患者于肝移植后第121天死亡,原因可能是肝-肺综合征。继发于GvHD的同种异体造血干细胞移植后可能发生器官衰竭,因此从单倍供体进行造血干细胞移植可能比从匹配的非亲属供体进行器官移植更有利。
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Combined Haploidentical Hematopoetic Stem Cell Transplantation and Liver Transplantation in a Pediatric Patient
Solid organ transplantation from the same donor is an established procedure for end-stage organ failure that developed after a previous hematopoietic stem cell transplantation (HSCT); however, it is rarely done in patients transplanted with unmanipulated haplo-HSCT. There are no pediatric reports regarding the long-term performance of organ transplantation after haplo-HSCT with post-transplant cyclophosphamide (PTCY). A juvenile myelomonocytic leukemia patient, who underwent unmanipulated haplo-HSCT with PTCY from her mother at the age of 3 years, developed chronic liver graft versus host disease (GvHD) which was refractory to specific GvHD treatment. Liver transplantation (LT) from her mother (the donor of her haplo-HSCT) was decided as the next line of treatment. LT was performed on day 540 post-HSCT, and the donor's left lateral segment was appropriately removed and attached to the recipient. The symptoms of GvHD completely regressed in a month. The patient died on day 121 after LT, because of a possible hepato-pulmonary syndrome. Organ failure can develop after allo-HSCT secondary to GvHD and therefore performing HSCT from a haplo-donor may be superior to a matched unrelated donor in terms of subsequent organ transplantation for organ failure.
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