Anemia aplásica. Hemoglobinuria paroxística nocturna

  1. Salido Fierrez, E.
  2. Cabañas Perianes, V.
  3. Moraleda Jiménez, J.M.
Journal:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Year of publication: 2016

Issue Title: Enfermedades de la sangre (I) Enfermedades de los hematíes

Series: 12

Issue: 20

Pages: 1159-1169

Type: Article

DOI: 10.1016/J.MED.2016.10.004 DIALNET GOOGLE SCHOLAR

More publications in: Medicine: Programa de Formación Médica Continuada Acreditado

Abstract

Abstract:Introduction The bone marrow failure syndromes are a heterogeneous group of clinical disorders pathophysiologically different and characterized by failure in normal hematopoietic function of the bone marrow. Clasification They are classified in: congenital (Fanconi anemia and Blackfand-Diamond anemia) or acquired, affecting selectively blood cell lineages (erythroblastopenia, leucopenia or thrombocytopenia) or impairing the hematopoiesis globally. The risk of morbidity and death is significant due to their progressive natural history, the risk of clonal evolution and complications of suboptimal therapy. It is not surprising that these conditions overlap in their presentation making undistinguishable in the early stages. Bone marrow failure syndromes In this chapter, aplastic anemia (AA), the most representative bone marrow failure syndrome, and paroxysmal nocturnal hemoglobinuria (PNH), will be described. Both are bone marrow failure syndromes but are clinical and pathophysiologically different. Clinically, AA overlaps with PNH in one patient, and PNH can evolve from a AA.

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