Paternal Split‐Liver Transplantation Followed by Haploidentical Hematopoietic Cell Transplantation in an Adult Patient With Protoporphyria‐Induced Liver Failure
Year: 2025
Type: article
Study design: Case Report
Abstract: 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.
Source: eJHaem
DOI: 10.1002/jha2.1092
Institutions: Klinikum Chemnitz, Christian-Albrechts-Universität zu Kiel, University Hospital Schleswig-Holstein, University Hospital Leipzig, University Hospital Carl Gustav Carus, Technische Universität Dresden, Beth Israel Deaconess Medical Center, Harvard University, Johannes Gutenberg University Mainz
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,860
OpenAlex ID: W4407676698