Hemoglobinuria paroxística nocturna / Paroxysmal nocturnal hemoglobinuria
Med. leg. Costa Rica
;
34(1): 242-252, ene.-mar. 2017.
Article
in Spanish
| LILACS
| ID: biblio-841447
RESUMEN
ResumenLa hemoglobinuria paroxística nocturna es una anemia hemolítica crónica, adquirida, poco común, que afecta con igual frecuencia ambos sexos. Se manifiesta a cualquier edad y con mayor incidencia en países del sudeste asiático. Es el resultado de la expansión clonal no maligna de células progenitoras hematopoyéticas. Se caracteriza por anemia hemolítica intravascular, tendencia a la trombosis y un componente variable de insuficiencia medular.Se asocia a otras patologías hematológicas como anemia aplásica y síndrome mielodisplásico. La citometría de flujo es el método de elección para diagnóstico. El eculizumab y el trasplante de médula ósea alogénico son las únicas terapias efectivas.
ABSTRACT
AbstractParoxysmal 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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Proteinuria
/
Hemoglobinuria, Paroxysmal
Limits:
Humans
/
Male
Country/Region as subject:
Central America
/
Costa Rica
Language:
Spanish
Journal:
Med. leg. Costa Rica
Journal subject:
Jurisprudence
Year:
2017
Type:
Article
Affiliation country:
Costa Rica
Institution/Affiliation country:
CCSS/CR
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