Reappraisal of liver transplantation for erythropoietic protoporphyria: A deadly combination of disease recurrence and biliary complication.
bile leakage
disease recurrence
erythropoietic protoporphyria
postoperative management
split liver transplantation
Journal
Pediatric transplantation
ISSN: 1399-3046
Titre abrégé: Pediatr Transplant
Pays: Denmark
ID NLM: 9802574
Informations de publication
Date de publication:
06 2022
06 2022
Historique:
revised:
23
01
2022
received:
03
09
2021
accepted:
17
02
2022
pubmed:
1
3
2022
medline:
12
5
2022
entrez:
28
2
2022
Statut:
ppublish
Résumé
Erythropoietic protoporphyria (EPP) is a rare inherited disorder that causes the accumulation of protoporphyrin in the erythrocytes, skin, and liver. Severe protoporphyric hepatopathy results in liver failure, requiring both liver and bone marrow transplantation as a life-saving procedure and to correct the underlying enzymatic defect, respectively. We report a 20-year-old man who underwent split liver transplantation using a right trisegment and caudate lobe graft for EPP-induced liver failure, but succumbed to a deadly combination of early relapse of EPP and subsequent, intractable, late-onset bile leakage from the cut surface of segment 4. EPP recurrence most likely created a high-risk situation for bile leakage from the non-communicating bile ducts of segment 4; therefore, this case shed light on the potential relationship between EPP recurrence and biliary complications. Physicians should recognize the potentially rapid and life-threatening progression of protoporphyric hepatopathy that leads to liver failure. For young patients with EPP, LT and sequential BMT should thoroughly be considered by a multidisciplinary team as soon as hepatic reserve deterioration becomes evident. Split liver transplantation should preferably be avoided and appropriate post-transplant management is critical before protoporphyrin depositions to the bile duct and hepatocyte causes irreversible damage to the liver graft.
Sections du résumé
BACKGROUND
Erythropoietic protoporphyria (EPP) is a rare inherited disorder that causes the accumulation of protoporphyrin in the erythrocytes, skin, and liver. Severe protoporphyric hepatopathy results in liver failure, requiring both liver and bone marrow transplantation as a life-saving procedure and to correct the underlying enzymatic defect, respectively.
CASE PRESENTATION
We report a 20-year-old man who underwent split liver transplantation using a right trisegment and caudate lobe graft for EPP-induced liver failure, but succumbed to a deadly combination of early relapse of EPP and subsequent, intractable, late-onset bile leakage from the cut surface of segment 4. EPP recurrence most likely created a high-risk situation for bile leakage from the non-communicating bile ducts of segment 4; therefore, this case shed light on the potential relationship between EPP recurrence and biliary complications.
CONCLUSION
Physicians should recognize the potentially rapid and life-threatening progression of protoporphyric hepatopathy that leads to liver failure. For young patients with EPP, LT and sequential BMT should thoroughly be considered by a multidisciplinary team as soon as hepatic reserve deterioration becomes evident. Split liver transplantation should preferably be avoided and appropriate post-transplant management is critical before protoporphyrin depositions to the bile duct and hepatocyte causes irreversible damage to the liver graft.
Substances chimiques
Protoporphyrins
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
e14261Informations de copyright
© 2022 Wiley Periodicals LLC.
Références
Casanova-Gonzalez MJ, Trapero-Marugan M, Jones EA, Moreno-Otero R. Liver disease and erythropoietic protoporphyria: a concise review. World J Gastroenterol. 2010;16(36):4526-4531.
Holme SA, Anstey AV, Finlay AY, Elder GH, Badminton MN. Erythropoietic protoporphyria in the U.K.: clinical features and effect on quality of life. Br J Dermatol. 2006;155(3):574-581.
McGuire BM, Bonkovsky HL, Carithers RL Jr, et al. Liver transplantation for erythropoietic protoporphyria liver disease. Liver Transpl. 2005;11(12):1590-1596.
Wahlin S, Stal P, Adam R, et al. Liver transplantation for erythropoietic protoporphyria in Europe. Liver Transpl. 2011;17(9):1021-1026.
Singal AK, Parker C, Bowden C, Thapar M, Liu L, McGuire BM. Liver transplantation in the management of porphyria. Hepatology. 2014;60(3):1082-1089.
Hanaki T, Noda T, Eguchi H, et al. Successful liver transplantation for liver failure with erythropoietic protoporphyria by covering the operating theater lights with polyimide film: a case report. Transplant Proc. 2020;52(2):625-629.
Park PJ, Hwang S, Choi YI, et al. Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria. Clin Mol Hepatol. 2012;18(4):411-415.
Smiers FJ, Van de Vijver E, Delsing JP, et al. Delayed immune recovery following sequential orthotopic liver transplantation and haploidentical stem cell transplantation in erythropoietic protoporphyria. Pediatr Transplant. 2010;14(4):471-475.
Windon AL, Tondon R, Singh N, et al. Erythropoietic protoporphyria in an adult with sequential liver and hematopoietic stem cell transplantation: A case report. Am J Transplant. 2018;18(3):745-749.
Hashimoto K, Fujiki M, Quintini C, et al. Split liver transplantation in adults. World J Gastroenterol. 2016;22(33):7500-7506.
Hampe T, Dogan A, Encke J, et al. Biliary complications after liver transplantation. Clin Transplant. 2006;20(Suppl 17):93-96.
Anstey AV, Hift RJ. Liver disease in erythropoietic protoporphyria: insights and implications for management. Gut. 2007;56(7):1009-1018.
Nunomura. Prevention of manifestation of porphyrias in the field of digestive tract surgery: abnormal porphyrin metabolism and early diagnosis of acute intermittent porphyria. St Marianna Med J. 1996;24(5):593-601.
Al-Samkari H, Patel AA, Schiano TD, Kuter DJ. Recurrence of acute intermittent porphyria after liver transplantation. Ann Intern Med. 2019;170(12):904-905.
Saruwatari H, Ueki Y, Yotsumoto S, et al. Genetic analysis of the ferrochelatase gene in eight Japanese patients from seven families with erythropoietic protoporphyria. J Dermatol. 2006;33(9):603-608.
Hackl C, Schmidt KM, Süsal C, Döhler B, Zidek M, Schlitt HJ. Split liver transplantation: current developments. Split Liver Transplantation: Current Developments. 2018;24(47):5312-5321.
Soyama A, Eguchi S, Egawa H. Liver transplantation in Japan. Liver Transpl. 2016;22(10):1401-1407.
Doyle MB, Maynard E, Lin Y, et al. Outcomes with split liver transplantation are equivalent to those with whole organ transplantation. J Am Coll Surg. 2013;217(1):102-112; discussion 113-104.
Sepulveda A, Scatton O, Tranchart H, et al. Split liver transplantation using extended right grafts: the natural history of segment 4 and its impact on early postoperative outcomes. Liver Transpl. 2012;18(4):413-422.