Open AccessPAROXYSMAL NOCTURNAL HEMOGLOBINURIA AND LIVER TRANSPLANTATION, A NEW PARADIGM.
Author(s) -
José Antonio Fernández,
Ana Sánchez Martínez,
José Antonio Pons
Publication year - 2020
Publication title -
revista española de enfermedades digestivas/revista española de enfermedades digestivas
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.331
H-Index - 38
eISSN - 2340-4167
pISSN - 1130-0108
DOI - 10.17235/reed.2020.7167/2020
Subject(s) - paroxysmal nocturnal hemoglobinuria , medicine , liver transplantation , hemoglobinuria , transplantation , gastroenterology , anemia
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.