EJHaem · 2025
Paternal Split-Liver Transplantation Followed by Haploidentical Hematopoietic Cell Transplantation in an Adult Patient With Protoporphyria-Induced Liver Failure.
СтатьяНазвание и аннотация: Перевод в очереди
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Stölzel Ulrich, Jost Lasse, Seehofer Daniel, Egger-Heidrich Katharina, Scheuermann Uwe, Hölig Kristina, Stauch Thomas, Kunadt Desiree, Schuppan Detlef, Schetelig Johannes, Wohmann Nils, Bornhäuser Martin, Stölzel Friedrich
Аннотация
INTRODUCTION: Erythropoietic Protoporphyria (EPP) caused skin light sensitivity and liver cirrhosis in a 35-year-old patient who subsequently developed liver-failure.
METHODS: In absence of a human leukocyte antigens (HLA)-matched-unrelated donor, the father consented in donating for split liver transplantation (SLT) and allogeneic hematopoietic cell transplantation (HCT).
RESULTS: After bridging therapy and successful SLT a first paternal HCT resulted in graft failure. For a second haploidentical HCT a different regimen was applied leading to engraftment while protoporphyrin (PP) blood-levels decreased to normal and skin light sensitivity skin disappeared, leading to complete remission in an immunosuppressive-free patient.
CONCLUSION: Haploidentical transplantation is a feasible treatment approach in EPP-patients.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.
METHODS: In absence of a human leukocyte antigens (HLA)-matched-unrelated donor, the father consented in donating for split liver transplantation (SLT) and allogeneic hematopoietic cell transplantation (HCT).
RESULTS: After bridging therapy and successful SLT a first paternal HCT resulted in graft failure. For a second haploidentical HCT a different regimen was applied leading to engraftment while protoporphyrin (PP) blood-levels decreased to normal and skin light sensitivity skin disappeared, leading to complete remission in an immunosuppressive-free patient.
CONCLUSION: Haploidentical transplantation is a feasible treatment approach in EPP-patients.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.