Paternal Split‐Liver Transplantation Followed by Haploidentical Hematopoietic Cell Transplantation in an Adult Patient With Protoporphyria‐Induced Liver Failure
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 li...
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| Main Authors: | , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2025-02-01
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| Series: | eJHaem |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/jha2.1092 |
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| Summary: | 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 |
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| ISSN: | 2688-6146 |