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

Dr. Alessandro Bucalossi, Divisione di Ematologia, ‘A. Sclavo’ Hospital, Siena, Via Tufi, 1, I-53100 Siena (Italy) 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 patients by Muller et al. [2]. We observed a similar condition in a 19-year-old man referred to our hospital for a serious anemia. A complete blood count showed: Hb, 4 g/dl; MCV, 94 fl; reticulocyte count, 0.2%; WBC, 3.5 × 109/1; platelet count, 247 × 109/1. Bone marrow histologic and cytologic examination displayed a hy-percellular picture with dysplastic erythro-poietic hyperplasia, characterized by maturation arrest at the level of proerythroblasts with rare basophilic erythroblasts, nuclear-cytoplasmic asynchrony, and megaloblastic features. Mild maturation defects were also present in both granulocytic and megakaryo-cytic lineages, with a blast count less than 5%. No chromosomal abnormality was detected. Moreover, there was no serological evidence for parvovirus B19 infection. A diagnosis of refractory anemia was made after other causes of anemia had been excluded. Therapy with erythropoietin was started for 4 weeks (200 U/kg, three times weekly) without any effect on the transfusion frequency. According to previous reports that described response to glucocorticoid therapy in myelodysplastic syndromes (3, 4), we began treatment with high-dose metyhl-prednisolone (2g/l2h, 3 times). The same schedule was repeated after 1 week and followed by prednisone (1.5 mg/kg/day) gradually tapered off and withdrawn once the Hb level achieved a normal value. A bone marrow aspirate performed at this time showed an almost complete resolution of dyserythro-poiesis 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 characterized by a maturation block of erythro-poiesis, normal karyotype, good response to immunosuppressive treatment and perhaps liable to spontaneous remission. In this regard, it may be appropriate to distinguish this last subset of patients with dyserythro-poiesis from clonal myelodysplastic syndromes and to consider it as one of the ‘autoimmune myelodysplasias’ according to Mieschner et al. [5]. References