Paternal Split-Liver Transplantation Followed by Haploidentical Hematopoietic Cell Transplantation in an Adult Patient With Protoporphyria-Induced Liver Failure

被引：0

作者：

Stoelzel, Ulrich ^{[1
,2
]}

Jost, Lasse ^{[3
]}

Seehofer, Daniel ^{[4
]}

Egger-Heidrich, Katharina ^{[5
]}

Scheuermann, Uwe ^{[4
]}

Hoelig, Kristina ^{[5
]}

Stauch, Thomas ^{[6
]}

Kunadt, Desiree ^{[5
]}

Schuppan, Detlef ^{[7
,8
]}

Schetelig, Johannes ^{[5
]}

Wohmann, Nils ^{[1
,2
]}

Bornhaeuser, Martin ^{[5
]}

Stoelzel, Friedrich ^{[3
,5
]}

机构：

[1] Klinikum Chemnitz, Dept Internal Med 2, Chemnitz, Germany

[2] Klinikum Chemnitz, Porphyria Ctr, Chemnitz, Germany

[3] Univ Kiel, Univ Hosp Schleswig Holstein Kiel, Dept Internal Med 2, Div Stem Cell Transplantat & Cellular Immunotherap, Kiel, Germany

[4] Univ Hosp Leipzig, Dept Visceral Transplantat Vasc & Thorac Surg, Leipzig, Germany

[5] Tech Univ Dresden, Univ Hosp Carl Gustav Carus, Dept Internal Med 1, Dresden, Germany

[6] MVZ Lab Volkmann & Kollegen GbR, Deutsch Kompetenz Zentrum Porphyriediagnost & Kons, Karlsruhe, Germany

[7] Johannes Gutenberg Univ Mainz, Inst Translat Immunol, Mainz, Germany

[8] Harvard Med Sch, Beth Israel Deaconess Med Ctr, Div Gastroenterol, Boston, MA USA

来源：

EJHAEM | 2025年 / 6卷 / 01期

关键词：

haploidentical; liver disease; porphyria; stem cell transplantation; ERYTHROPOIETIC PROTOPORPHYRIA;

D O I：

10.1002/jha2.1092

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

IntroductionErythropoietic Protoporphyria (EPP) caused skin light sensitivity and liver cirrhosis in a 35-year-old patient who subsequently developed liver-failure.MethodsIn 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).ResultsAfter 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.ConclusionHaploidentical transplantation is a feasible treatment approach in EPP-patients.Trial RegistrationThe authors have confirmed clinical trial registration is not needed for this submission

引用

页数：4

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