Paroxysmal nocturnal hemoglobinuria: a rare acquired hematologic disorder.
Mónica Santiago Casiano, Liza M Paulo Malavé, Omayra González Rodríguez, William Cáceres
Abstract
Mónica Santiago Casiano, Liza M Paulo Malavé, Omayra González Rodríguez, William Cáceres
Abstract
Paroxysmal nocturnal hemoglobinuria is a rare hematological disorder. It is an uncommon cause of intravascular hemolysis, thrombosis and bone marrow suppression. We report a 19-year-old female patient admitted to the hospital with pancytopenia. Workout of pancytopenia disclosed paroxysmal nocturnal hemoglobinuria. The patient responded well to treatment with blood transfusions, steroids and eculizumab. We discuss the underlying pathophysiology, clinical manifestations and treatment of this rare entity.
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Paroxysmal nocturnal hemoglobinuria is a rare hematological disorder. It is an uncommon cause of intravascular hemolysis, thrombosis and bone marrow suppression. We report a 19-year-old female patient admitted to the hospital with pancytopenia. Workout of pancytopenia disclosed paroxysmal nocturnal hemoglobinuria. The patient responded well to treatment with blood transfusions, steroids and eculizumab. We discuss the underlying pathophysiology, clinical manifestations and treatment of this rare entity.
Key concepts: Paroxysmal nocturnal hemoglobinuria, Pancytopenia, Eculizumab, Medicine, Hemoglobinuria, Bone marrow, Bone marrow failure, Hemolysis