Paroxysmal nocturnal hemoglobinuria and liver transplantation, a new paradigm.

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA AND LIVER TRANSPLANTATION, A NEW PARADIGM. Rev Esp Enferm Dig. 2020 Nov 24;: Authors: Morilla Fernandez JA, Sánchez Martínez A, Pons Miñano JA 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, the 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 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. Our patient is a 53-year-old man, with no relevant medical history, diagnosed in 1993 with hemolytic anemia (Coombs-, Ham-) and discharged with immunosuppressive and corticosteroid therapy, who was unable to suspend due to repeated hemolytic crisis. In 2004, abdominal ultrasound and abdominal CT were performed due to dyspeptic symptoms, showing stigmas of chronic liver disease, signs of portal hypertension and portal cavernomatosis secondary to Budd-Chiari syndrome. In 2006, the first clonality study was carried out, being conclusive with PHN. In January 2009, treatment with eculizumab was started and disease...
Source: Revista Espanola de Enfermedades Digestivas - Category: Gastroenterology Tags: Rev Esp Enferm Dig Source Type: research