Pure red cell aplasia, a disease of a great diversity

Abstract

Pure red cell aplasia (PRCA) is a type of normocytic or sometimes macrocytic anemia characterized by reticulocytopenia. The cause of hypo erythropoiesis is varied. It could be of a congenital or an acquired type. A congenital PRCA is different than anemia caused by congenital dyserythropoiesis. The classification of acquired PRCA can be primary when no cause is identified or secondary due to underling or associated pathology. The primary PRCA is a disease of exclusion. There are many disorders such as autoimmune disorders, hematological and nonhematological malignancies, some infections, and medications that cause secondary PRCA. Immune-mediated mechanism plays an important role in both primary and secondary types. Therefore, immunomodulating agents are important in the treatment of primary type and many secondary types. Other therapy modalities include surgery, plasmapheresis, and hemopoietic stem cell transplantation. The assessment of hemoglobin level and absolute reticulocyte count with the frequency of blood transfusion are important to monitor therapy response achievement. Achievement of complete response is important to eliminate demands for blood transfusion and to avoid blood-transfusion risks. Herein, we review this disorder which has broad diversity, its causes, approach to its cause, therapy modalities.

Keywords

• dyserythropoiesis
• pure red cells aplasia
• reticulocytopenia