The term pure red cell aplasia (PRCA) indicates a heterogeneous group of congenital or acquired blood disorders characterized by anemia, reticulocytopenia, and paucity of erythroid precursors in an otherwise normally cellular bone marrow. Some forms of PRCA appear to have an immune-mediated pathogenesis; both humoral inhibitors and T-cell mediated inhibition of erythropoiesis have been described. We describe the case of a child with immune-mediated PRCA who was successfully treated by high intravenous doses of gamma-globulin.

Case report: A 7-year-old girl was admitted to our Department for further investigation of anemia. The patient had been well until 1 month earlier, when she began to have anorexia and fatigue. At entry she had severe normochromic, normocytic anemia with hemoglobin of 70 g/L and reticulocyte count was 1 × 109/L. Bone marrow examination revealed a cellular marrow with a marked decrease in erythroid precursors. There was no serologic evidence of Epstein-Barr virus, human cytomegalovirus, or hepatites virus infection. No antibodies to parvovirus B19 were found. Hematopoietic colony cultures demonstrated the existence of a humoral factor inhibiting the erythroid progenitors, as described in some patients with acquired erythroblastopenia.

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