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Medicina Legal de Costa Rica
On-line version ISSN 2215-5287Print version ISSN 1409-0015
Abstract
GARCIA ROJAS, Víctor Andrés; SANCHEZ VARGAS, Xinia Alexandra and MORA HERNANDEZ, Gustavo Adolfo. Hemoglobinuria paroxística nocturna. Med. leg. Costa Rica [online]. 2017, vol.34, n.1, pp.342-352. ISSN 2215-5287.
Paroxysmal nocturnal hemoglobinuria is a rare acquired chronic hemolytic anemia, which affects both sexes with equal frequency. It occurs at any age and more frequently in Southeast Asian countries. It is the result of non malignant clonal expansion of hematopoietic progenitor cells. It is characterized by intravascular hemolytic anemia, recurrent thrombosis and a variable component of bone marrow failure. It is associated with other hematologic disorders such as aplastic anemia and myelodysplastic syndrome. Flow cytometry is the method of choice for diagnosis. Eculizumab and allogeneic bone marrow transplantation is the only effective therapies.
Keywords : Paroxysmal nocturnal hemoglobinuria; Microangiopathic hemolytic anemia.