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Paroxysmal nocturnal hemoglobinuria: a rare acquired hematologic disorder.

Mónica Santiago Casiano, Liza M Paulo Malavé, Omayra González Rodríguez, William Cáceres

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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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What this paper is about

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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Available 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.

Key concepts: Paroxysmal nocturnal hemoglobinuria, Pancytopenia, Eculizumab, Medicine, Hemoglobinuria, Bone marrow, Bone marrow failure, Hemolysis

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