{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T22:16:29Z","timestamp":1782252989111,"version":"3.54.5"},"reference-count":27,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,6,2]],"date-time":"2021-06-02T00:00:00Z","timestamp":1622592000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>EPP is characterized by photosensitivity and by liver disease. When LT is performed in EPP, recurrence often occurs in the allograft due to ongoing protoporphyrin production in bone marrow. Therefore, curative treatment requires allogeneic HSCT after LT. Long\u2010term immunosuppression could be spared by using the same donor for both transplants.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A 2\u2010year\u2010old girl with EPP in liver failure underwent liver transplant from her father. Transfusion and apheresis therapy were used to lower protoporphyrin levels before and after liver transplant. Ten weeks after liver transplant, she underwent HSCT, using the same donor. Conditioning was with treosulfan, fludarabine, cyclophosphamide, and ATG. GVHD prophylaxis was with abatacept, methotrexate, MMF, and tacrolimus. We followed the patient's erythrocyte protoporphyrin and liver and skin health for 2\u00a0years after transplant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>After hematopoietic stem cell engraftment, a decline in protoporphyrin levels was observed, with clinical resolution of photosensitivity. Liver biopsies showed no evidence of EPP. Mild ACR occurred and responded to steroid pulse. Two years post\u2010HSCT, the patient has been weaned off all immunosuppression and remains GVHD and liver rejection free.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Sequential liver and HSCT from the same haploidentical donor are feasible in EPP. This strategy can allow for discontinuation of immune suppression.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14040","type":"journal-article","created":{"date-parts":[[2021,6,2]],"date-time":"2021-06-02T18:01:59Z","timestamp":1622656919000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Sequential paternal haploidentical donor liver and HSCT in EPP allow discontinuation of immunosuppression post\u2010organ transplant"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5465-6169","authenticated-orcid":false,"given":"Sarah","family":"Malkiel","sequence":"first","affiliation":[{"name":"Division of Hematology\/Oncology Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Blayne A.","family":"Sayed","sequence":"additional","affiliation":[{"name":"Division of General and Thoracic Surgery Hospital for Sick Children Toronto  ON Canada"},{"name":"Department of Surgery University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9998-5692","authenticated-orcid":false,"given":"Vicky","family":"Ng","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Department of Paediatrics, Transplant and Regenerative Medicine Centre Hospital for Sick Children University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donna A.","family":"Wall","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacob","family":"Rozmus","sequence":"additional","affiliation":[{"name":"Division of Oncology, Hematology and BMT Department of Pediatrics BC Children's Hospital\/University of British Columbia  Vancouver BC Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard A.","family":"Schreiber","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Department of Pediatrics Faculty of Medicine University of British Columbia  Vancouver BC Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Farah","family":"Faytrouni","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Department of Pediatrics Faculty of Medicine University of British Columbia  Vancouver BC Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iram","family":"Siddiqui","sequence":"additional","affiliation":[{"name":"Department of Pathology Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kuang\u2010Yueh","family":"Chiang","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yaron","family":"Avitzur","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Department of Paediatrics, Transplant and Regenerative Medicine Centre Hospital for Sick Children University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,6,2]]},"reference":[{"key":"e_1_2_10_2_1","volume-title":"GeneReviews","author":"Balwani M","year":"2014"},{"key":"e_1_2_10_3_1","first-page":"1009","article-title":"Liver disease in erythropoietic protoporphyria: insights and implications for management","volume":"56","author":"Anstey AV","year":"2007","journal-title":"Gut"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.20620"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.22341"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2006-0833"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2009.01233.x"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2009.12.413"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2019.12.530"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.14581"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2006.10.004"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1155\/2016\/6471901"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V96.12.3997.h8003997_3997_3999"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0000000000002649"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.21527"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200002033420504"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2002.120460"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa071074"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1983.tb07681.x"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01535955"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2004.04190.x"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007890-199903270-00023"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9272-6"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2015.08.004"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1053\/bbmt.2001.v7.pm11787529"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1702151"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2017.1557"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.00505.2004"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.14040","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/petr.14040","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.14040","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T15:30:35Z","timestamp":1693323035000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.14040"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,2]]},"references-count":27,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2021,9]]}},"alternative-id":["10.1111\/petr.14040"],"URL":"https:\/\/doi.org\/10.1111\/petr.14040","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,2]]},"assertion":[{"value":"2021-02-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-04-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-06-02","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e14040"}}