Pure Red Cell Aplasia

  • Sanford B. Krantz
  • S. Donald Zaentz


Pure red cell aplasia (PRCA) was first described by Kaznelson (1922) as an anemia due to an almost complete cessation of erythropoiesis, but without leukopenia or thrombocytopenia. This condition usually can be clearly distinguished from aplastic anemia in which low blood white cell and platelet concentrations accompany the anemia (Van Der Weyden and Firkin, 1972). In PRCA the bone marrow has an isolated absence of erythroblasts with normal granulocytopoiesis and megakaryocytopoiesis (DiGiacomo et al., 1966; Jacobs et al., 1959), whereas in aplastic anemia all three hematopoietic lineages are markedly reduced (Van Der Weyden and Firkin, 1972). In children PRCA has been called “chronic (congenital) hypoplastic anemia,” the Diamond-Blackfan anemia, or erythrogenesis imperfecta (Diamond and Blackfan, 1938; Gasser, 1957; Tsai and Levin, 1957). In adults it has been termed isolated aplastic anemia, aplastic crisis, erythroblastopenia, erythrophthisis, chronic erythrocytic hypoplasia, red cell aplastic anemia, or red cell agenesis (Gasser, 1957; Tsai and Levin, 1957). Although these cases could represent a variety of pathogenetic mechanisms they all have in common an isolated absence of marrow erythroblasts and are now considered together as cases of PRCA.


Chronic Lymphocytic Leukemia Marrow Cell Refractory Anemia Heme Synthesis Aplastic Crisis 
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Copyright information

© Springer Science+Business Media New York 1977

Authors and Affiliations

  • Sanford B. Krantz
    • 1
  • S. Donald Zaentz
    • 1
  1. 1.Division of Hematology, Departments of MedicineVanderbilt University School of Medicine and Veterans Administration HospitalNashvilleUSA

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