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Acquired pure red cell aplasia in the adult

INTRODUCTION

Acquired pure red cell aplasia (PRCA) is a rare, generally chronic condition of profound anemia characterized by a severe reduction in the number of reticulocytes in the peripheral blood and the virtual absence of erythroid precursors in the bone marrow. All other cell lineages are present and appear morphologically normal.

A similar but usually self-limited condition seen during the first years of life, transient erythroblastopenia of childhood, is discussed separately. (See "Anemia in children due to decreased red blood cell production", section on 'Transient erythroblastopenia of childhood (TEC)'.)

The congenital form of the disease is known as Diamond-Blackfan anemia/syndrome, and is associated with a number of congenital abnormalities [1,2]. (See "Anemia in children due to decreased red blood cell production", section on 'Diamond-Blackfan anemia'.)

Acquired PRCA will be discussed here. Acquired PRCA due to the presence of anti-erythropoietin antibodies secondary to treatment with recombinant human erythropoietin is discussed separately. (See "Pure red cell aplasia due to anti-erythropoietin antibodies".)

ETIOLOGY AND PATHOGENESIS

Associated disorders — Acquired pure red cell aplasia (PRCA) in the adult is most often idiopathic, although a number of underlying causes can exist (table 1) [1,3].

                  

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Literature review current through: Sep 2014. | This topic last updated: Nov 5, 2013.
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