title: Late-onset graft-versus-host disease after pediatric living-related liver transplantation for Langerhans cell histiocytosis.
abstract: GVHD is the most common and well-known cause of morbidity and mortality following allogeneic BM transplantation. The GVHD following OLT is an uncommon complication but has a high mortality and poses a major diagnostic and therapeutic challenge. We herein discussed a 12-month-old girl with multi-system LCH, who developed end-stage liver disease despite intensive chemotherapy. She underwent ABO-compatible liver transplantation at 28 months while in remission from LCH. The donor was her 26-yr-old father. Post-operative course was uneventful. The GVHD manifested with skin rash and BM suppression on post-transplant day 94 and confirmed by both microchimerism and skin biopsy. Prednisolone, basiliximab, and ATG were administered immediately but the bone marrow suppression was not improved and the patient died because of Candida sepsis at six-month post-transplant. GVHD after OLT should be keep in mind in patients with rash and BM suppression after liver transplantation. In LDLT, a patient who carries risk factors should investigated for optimal HLA matching.
authors: Department of Pediatric Gastroenterology, Ege University School of Medicine, Izmir, Turkey.
authors: Hasan Ali
authors: HA
authors: Yuksekkaya
authors: Cigdem
authors: C
authors: Arikan
authors: Gokhan
authors: G
authors: Tumgor
authors: Serap
authors: S
authors: Aksoylar
authors: Murat
authors: M
authors: Kilic
authors: Sema
authors: S
authors: Aydogdu
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D000077552:Basiliximab; D001706:Biopsy; D016026:Bone Marrow Transplantation / Q000379:methods; D058625:End Stage Liver Disease / Q000628:therapy; D004890:Erythema / Q000175:diagnosis; D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis*; D006680:HLA Antigens / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007801:Langerhans Cells / Q000166:cytology*; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D010641:Phenotype; D011239:Prednisolone / Q000627:therapeutic use; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use
chemical_list: D000911:Antibodies, Monoclonal; D000961:Antilymphocyte Serum; D006680:HLA Antigens; D011993:Recombinant Fusion Proteins; D000077552:Basiliximab; D011239:Prednisolone