Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant

Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant

ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH,...

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Bibliographic Details
Main Authors: Railene Célia B. Alencar, Andréa M. Guimarães, Lacy C. Brito Junior
Format: Article
Language:English
Published: Sociedade Brasileira de Patologia Clínica
Series:Jornal Brasileiro de Patologia e Medicina Laboratorial
Subjects:
pure red-cell aplasia
paroxysmal hemoglobinuria
liver transplantation
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442016000500307&lng=en&tlng=en
Description
Summary:ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of PNH with eculizumab. Clinical stabilization and cessation of symptoms were achieved.
ISSN:1676-2444
1678-4774

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