Anemias por fallo medular. Anemia aplásica adquirida. Hemoglobinuria paroxística nocturna

  1. García Suárez, J. 1
  2. Conde Royo, D. 1
  1. 1 Servicio de Hematología y Hemoterapia. Hospital Universitario Príncipe de Asturias. Alcalá de Henares. Madrid. España
Revista:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Ano de publicación: 2020

Serie: 13

Número: 21

Páxinas: 1178-1190

Tipo: Artigo

DOI: 10.1016/J.MED.2020.11.011 DIALNET GOOGLE SCHOLAR

Outras publicacións en: Medicine: Programa de Formación Médica Continuada Acreditado

Obxectivos de Desenvolvemento Sustentable

Resumo

Acquired aplastic anemia is a rare disorder which causes bone marrow failure syndrome due to the autoimmune destruction of the hematopoietic stem cells (HSC). New molecular and genetic methods have improved knowledge of the pathogenesis of aplastic anemia, finding somatic mutations, cytogenetic anomalies, and defective telomere replication. These advances in regard to the physiopathological mechanisms allow for improving treatment strategies (including allogenic hematopoietic stem cell transplantation), increasing survival, and personalized treatment in the future. Paroxysmal nocturnal hemoglobinuria is a rare clonal disease of the HSC with a wide variety of symptoms. It is characterized by the presence of hemolytic anemia, thrombosis, and a certain degree of bone marrow failure. New anticomplement therapies such as eculizumab represent a revolution in the management of this disease.

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Fonte de datos: Dialnet