[Acquired pure red cell aplasia].

Abstract

Acquired pure red cell aplasia (PRCA), characterized by anemia, reticulocytopenia, and erythroid hypoplasia in the marrow, is a bone marrow failure syndrome that mainly affects elderly adults. Underlying T-cell dysregulations, often associated with clonality and/or STAT3 gene mutation, have been a rationale for using cyclosporin and other directed immunosuppressive therapies in most of the disease subtypes, namely, thymoma-associated PRCA, large granular lymphocytic leukemia-associated PRCA, and idiopathic PRCA. Although the epidemiologic rarity of PRCA has precluded comparative clinical trials that could demonstrate the superiority of one immunosuppressive agent over another, some retrospective studies have demonstrated the significance of maintenance therapies to avoid blood transfusion dependency, a factor that may lead to poorer prognosis in patients with PRCA. This review primarily discusses clinical aspects of PRCA in line with recently updated clinical guidelines.