Paroxysmal nocturnal hemoglobinuria: a rare acquired hematologic disorder
Santiago Casiano, M.ón.; Paulo Malavé, L.M.; González Rodríguez, O.; Cáceres, W.
Boletin de la Asociacion Medica de Puerto Rico 103(3): 30-33
2011
ISSN/ISBN: 0004-4849 PMID: 23210330 Document Number: 702542
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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