Graft-vs.-host disease (GVHD) is a rare, serious complication of orthotopic liver transplantation (OLT). We have treated 5 patients to date with GVHD after OLT. A total of 78 patients worldwide have been reported to have experienced this complication. The means by which GVHD after OLT has been managed is guided by experience with the more common GVHD that occurs after stem cell transplantation. However, despite the use of various treatment modalities, the mortality of GVHD after OLT remains high. This case series and review of the literature demonstrates that successful resolution of GVHD after OLT cannot be expected with the use of those modalities that have been tried to date. It is imperative that new treatments be applied to GVHD after OLT in order to improve the prognosis of patients with this diagnosis.
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http://dx.doi.org/10.1002/lt.21203 | DOI Listing |
Front Pediatr
March 2022
Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wrocław Medical University, Wrocław, Poland.
We report a child with Fanconi anemia who, after hematopoietic stem cell transplantation (HSCT) complicated by acute graft-versus-host disease (GVHD), underwent orthotopic liver transplantation (OLT). Approximately 1 month after OLT, the presence of third-party genetic material from the liver donor was noted and in the next few weeks, the chimerism assessment revealed 100% liver donor leukocytes in the peripheral blood. The rapidly progressing GVHD with gut involvement resulted in patient's death 6 months after OLT.
View Article and Find Full Text PDFMol Genet Metab Rep
March 2022
Laboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Department of Clinical Chemistry, Amsterdam Gastroenterology Endocrinology Metabolism, Amsterdam, the Netherlands.
We describe the case of a Greek female patient with the Classic form of the ultra- rare and fatal autosomal recessive disorder Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) and the impact of allogeneic hematopoietic stem cell transplantation on the biochemical and clinical aspects of the disease. The patient presented at the age of 15 years with severe gastrointestinal symptoms, cachexia, peripheral neuropathy and diffuse leukoencephalopathy. The diagnosis of MNGIE disease was established by the increased levels of thymidine and deoxyuridine in plasma and the complete deficiency of thymidine phosphorylase activity.
View Article and Find Full Text PDFLiver Transpl
March 2022
Division of HematologyDepartment of Medicine University of Washington Seattle WA Division of Transplant Surgery University of Washington Seattle WA Clinical and Bio-Analytics Transplant Laboratory in the Department of Surgery at the University of Washington School of Medicine Seattle WA Bloodworks Northwest Seattle WA Division of GastroenterologyDepartment of Medicine University of Washington Seattle WA Division of Gastroenterology, Hepatology, and Nutrition, Department of Medicine, Vanderbilt University Medical Center Nashville TN.
Acute graft-versus-host disease (GVHD) is a rare complication after orthotopic liver transplantation (OLT) that carries high mortality. We hypothesized that machine-learning algorithms to predict rare events would identify patients at high risk for developing GVHD. To develop a predictive model, we retrospectively evaluated the clinical features of 1938 donor-recipient pairs at the time they underwent OLT at our center; 19 (1.
View Article and Find Full Text PDFPediatr Transplant
September 2021
Department of Bone Marrow Transplantation and Cellular Therapy, St. Jude Children's Research Hospital, Memphis, TN, USA.
Background: EPP is a rare disorder of heme biosynthesis in which patients present with disabling photosensitivity. A subset of patients develop severe liver disease with progressive liver failure necessitating an OLT. A HCT can potentially cure EPP by replacing the native bone marrow, which is the primary site of heme synthesis.
View Article and Find Full Text PDFTranspl Immunol
June 2019
Medanta Institute of Liver Transplantation and Regenerative Medicine, Medanta-the Medicity, Gurugram, India. Electronic address:
Graft versus Host Disease (GVHD) after orthotopic liver transplant (OLT), although rare, carries >80% mortality. Early diagnosis, prompt treatment, and aggressive supportive care are imperative to potentially reverse this otherwise fatal ailment. We describe a case of severe diarrhoea post living donor OLT who was diagnosed with acute GVHD.
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