Year 2021 / Volume 113 / Number 4
Letter
Paroxysmal nocturnal hemoglobinuria and liver transplantation, a new paradigm

303-304

DOI: 10.17235/reed.2020.7167/2020

José Antonio Morilla Fernández, Ana Sánchez Martínez, José Antonio Pons Miñano,

Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a type of hemolytic anemia acquired by the PIG-A gene mutation. This causes a deficiency of a complement regulatory protein, CD59, which results in hemolysis, hemoglobinuria and thrombosis (due to the release of procoagulant factors). Budd-Chiari syndrome is characteristic in these patients and has classically been considered as a contraindication for liver transplantation (LT) due to post-transplant recurrence. Since the approval of eculizumab for the treatment of PHN, disease control is possible and therefore the post-transplant recurrence of thrombotic phenomena involving the liver is avoided.
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References
Ana Villegas, Beatriz Arrizabalaga Amuchastegui, Santiago Bonanad Boix et al. Consenso español para diagnóstico y tratamiento de Hemoglobinuria paroxística Nocturna. Med Clin. 2016; 146 (6): 278e1-7.
Muñoz-Linares, Emilio Ojeda, Rafael Forés et al. Paroxysmal nocturnal hemoglobinuria: a single Spanish center’s experience over the last 40 yr. Eur J Haematol. 2014; 93 (4): 309–19.
Singer AL, Locke JE, Stewart ZA, et al. Successful Liver Transplantation for Budd-Chiari Syndrome in a Patient with Paroxysmal Nocturnal Hemoglobinuria Treated with the Anti-Complement Antibody Eculizumab. Liver Transpl. 2009; 15: 540-543.
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Morilla Fernández J, Sánchez Martínez A, Pons Miñano J. Paroxysmal nocturnal hemoglobinuria and liver transplantation, a new paradigm. 7167/2020


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Publication history

Received: 20/04/2020

Accepted: 24/04/2020

Online First: 24/11/2020

Published: 07/04/2021

Article Online First time: 218 days

Article editing time: 352 days


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