Role of oral steroids in the management of pure red cell aplasia in children

Objective: to determine the role of oral steroids in the management of pure red cell aplasia in children. study design: prospective descriptive study. place and duration of study: it was carried out in the department of hematology and transfusion medicine of the children's hospital and the institute of child health,lahore from june 2004 to december 2008. patients and methods: this study included 30 children of pure red cell aplasia (prca) up to 5 years of age who were diagnosed after complete clinical evaluation and a range of investigations including complete blood count,reticulocyte count and bone marrow examination. children having normochromic normocytic anemia,low reticulocyte count and absent erythroid activity in the marrow were diagnosed as pure red cell aplasia. study cases were divided in two groups,diamond blackfan anemia (dba) and transient erythroblastopenia of childhood (tec) on the basis of their age of presentation and presence of congenital anomalies. oral steroids,deltacortil with the dose of 1mg/kg/day in 3 divided doses were given to patients of diamond blackfan anemia for 4-6 weeks while spontaneous remission was observed in transient erythroblastopenia of childhood. results: out of 30 children of pure red cell aplasia,23(76.7%) were of diamond blackfan anemia and 7(23.3%) were of tec. 4 patients of diamond blackfan anemia and 1 of transient erythroblastopenia of childhood lost follow up. steroids response was seen in 9 (47.3%) patients of diamond blackfan anemia and 10 (52.7%) showed no response. spontaneous remission occurred in all the 6 patients of transient erythroblastopenia of childhood. conclusion: oral steroids are the drug of choice in diamond blackfan anemia while transient erythroblastopenia of childhood is associated with spontaneous remission.