RT Book, Section A1 Young, Neal S. A2 Kaushansky, Kenneth A2 Lichtman, Marshall A. A2 Prchal, Josef T. A2 Levi, Marcel M. A2 Press, Oliver W. A2 Burns, Linda J. A2 Caligiuri, Michael SR Print(0) ID 1121091855 T1 Pure Red Cell Aplasia T2 Williams Hematology, 9e YR 2015 FD 2015 PB McGraw-Hill Education PP New York, NY SN 9780071833004 LK hemonc.mhmedical.com/content.aspx?aid=1121091855 RD 2024/03/28 AB SUMMARYPure red cell aplasia is the diagnosis applied to isolated anemia secondary to failure of erythropoiesis. Cardinal findings are a low hemoglobin level combined with reticulocytopenia and absent or extremely infrequent marrow erythroid precursor. Historical names for pure red cell aplasia include erythroblast hypoplasia, erythroblastopenia, red cell agenesis, hypoplastic anemia, and aregenerative anemia. Aplastic anemia confers the same meaning, of course, but is applied to pancytopenia and an empty marrow (Chap. 35). Pure red cell aplasia was first separated from aplastic anemia by Kaznelson in 1922. The association of red cell aplasia and thymoma interested physicians in the 1930s and ultimately led to laboratory studies linking pure red cell aplasia to immune mechanisms, including the early identification of antierythroid precursor cell antibodies by Krantz and later characterization of T cells that inhibited erythropoiesis. Red cell aplasia as an acute and life-threatening complication of sickle cell disease and other hemolytic anemias was recognized in the 1940s, presaging the role of a specific virus in the etiology of both acute and chronic erythropoietic failure. Despite its infrequency, pure red cell aplasia has been a subject of much laboratory research because of its link to an immune mechanism of erythropoietic failure and as a manifestation of parvovirus B19 infection and viral destruction of red cell progenitors. However, because of its infrequency, pure red cell aplasia has not been the subject of large or controlled clinical trials; as a result, therapeutic recommendations are based on single cases or small series. Table 36–1 lists a practical classification of pure red cell aplasia.