Pure Red Cell Anemia with Erythropoietic Maturation Arrest and Spontaneous Recovery

Abstract

We report the case of a 21-year-old male who presented with severe anemia and hypercellular bone marrow, without known cause. The dominant feature of the bone marrow was that of a maturation arrest in the erythroid series, with absolute reticulocytopenia in the peripheral blood. Immunosuppressive therapy was withheld, but transfusions were given to maintain his hemoglobin prior to full recovery. The patient had features of both pure red cell anemia and of transient erythroblastopenia of childhood.

Successful Treatment of a Patient with Refractory Anemia by Immunosuppressive Therapy: Another Case of ‘Autoimmune Myelodysplasia’?

“…A bone mar row aspirate performed at this time showed an almost complete resolution of dyserythropoiesis with normal granulocytic and megakaryocytic maturation. In conclusion, the analysis of the cases reported in the literature [ 1,2], along with our experience, provides further evidence about a rare subset of young patients with dyserythropoietic anemia char acterized by a maturation block of erythropoiesis, normal karyotype, good response to immunosuppressive treatment and perhaps liable to spontaneous remission. In this re gard, it may be appropriate to distinguish this last subset of patients with dyserythropoiesis from clonal myelodysplastic syn dromes and to consider it as one of the 'autoimmune myelodysplasias' according to Mieschneret al [5].…”

“…We read with interest the report by Davis and Farver [1] concerning the spontaneous recovery of anemia in a young patient with an atypical pure red cell aplasia. This is probably a further description of a peculiar disease already reported in two young pa tients by Muller et al [2],…”