RT Book, Section A1 Young, Neal S. A2 Prchal, Josef T. A2 Lichtman, Marshall A. SR Print(0) ID 1184010245 T1 Pure Red Cell Aplasia T2 Williams Hematology The Red Cell and Its Diseases YR 2022 FD 2022 PB McGraw Hill PP New York, NY SN 9781264269075 LK hemonc.mhmedical.com/content.aspx?aid=1184010245 RD 2024/03/29 AB SUMMARYPure red cell aplasia is defined by the combination of anemia, reticulocytopenia, and the absence of erythroid precursors on the marrow examination, but the pathophysiologies are diverse. In children, a genetic defect in ribosome protein genes is responsible for Diamond-Blackfan anemia. In older adults, pure red cell aplasia is usually immune-mediated, sometimes associated with thymoma, and responsive to immunosuppressive therapies. The syndrome can overlap with other diseases, especially large granular lymphocytosis, or complicate them, as with chronic lymphocytic leukemia (CLL) or myelodysplastic syndrome. Parvovirus B19 is an infectious etiology, acutely producing transient aplastic crisis of hemolytic anemia and, when persistent, identical to pure red cell aplasia. The exclusive loss of erythrocyte production is a rare complication of medical drug therapy, but pure red cell aplasia can be iatrogenic, as with anti-erythropoietin antibodies after administration of the hormone and in ABO-incompatible allogeneic transplantation caused by persistence of host isoagglutinins.