title: Positive Impact of Eculizumab Therapy on Surgery for Budd-Chiari Syndrome in a Patient with Paroxysmal Nocturnal Hemoglobinuria and a Long-Term History of Thrombosis.
abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response. In October 2012, he developed upper gastrointestinal variceal bleeding and a splenorenal shunt was placed. At the time of writing, the patient remains stable and eculizumab continues to be effective. There is limited data on the use of eculizumab for prevention of hemolysis and its consequences in PNH patients undergoing surgery. Our findings provide evidence for the efficacy and safety of eculizumab in this setting.
authors.affiliation: Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
authors.forename: Silvia
authors.initials: S
authors.lastname: De-la-Iglesia
authors.affiliation: Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
authors.forename: Hugo
authors.initials: H
authors.lastname: Luzardo
authors.affiliation: Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
authors.forename: Angelina
authors.initials: A
authors.lastname: Lemes
authors.affiliation: Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
authors.forename: Melissa
authors.initials: M
authors.lastname: Torres
authors.affiliation: Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Gómez-Casares
authors.affiliation: Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
authors.forename: Naylen
authors.initials: N
authors.lastname: Cruz
authors.affiliation: Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
authors.forename: Teresa
authors.initials: T
authors.lastname: Molero
keywords: Budd-Chiari syndrome; Paroxysmal nocturnal haemoglobinuria (PNH); eculizumab; surgery; thrombosis