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.

Autor: De-la-Iglesia S; Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain., Luzardo H; Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain., Lemes A; Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain., Torres M; Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain., Gómez-Casares MT; Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain., Cruz N; Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain., Molero T; Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria , Spain.
Jazyk: angličtina
Zdroj: Hematology reports [Hematol Rep] 2016 Sep 30; Vol. 8 (3), pp. 6562. Date of Electronic Publication: 2016 Sep 30 (Print Publication: 2016).
DOI: 10.4081/hr.2016.6562
Abstrakt: 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.
Competing Interests: the authors declare no potential conflict of interest.
Databáze: MEDLINE
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