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.

We decided to administer gamma-globulins (Sandoglobulin) intravenously, 1 g/Kg of body weight per 1 day. The reticulocyte count increased from 1 to 100× 109/L on day 7 after therapy. Hemoglobin levels rose to 92 g/L on day 10 after the end of therapy. Intravenous administration of gamma-globulin was repeated monthly for another 3 times and was well tolerated.

A marrow examination performed 3 months after therapy showed a normal population of erythroblastic cells. Six months after discontinuation of treatment, the patient has a normal complete blood cell count.

Conclusions: We conclude that this case indicates that children with immune-mediated erythropoietic failure may be successfully treated with high doses of intravenous gamma-globulin.