In order to explore the diagnosis and therapy of Diamond Blackfan anemia (DBA), 
the clinical data of 45 cases of DBA admitted in our hospital from February 1994 
to July 2011 were analyzed retrospectively. The clinical characteristics, 
results of laboratory examination, treatment reaction and outcome of disease 
were investigated. The results indicated that out of 45 children diagnosed as 
DBA, 14 cases (31.1%) had short stature and physical malformation. All patients 
had anemia with reticulocytopenia. Thirty-four patients (75.6%) had mean 
corpuscular volume. Eleven patients (24.4%) had macrocytic anemia. Bone marrow 
examination showed a marked erythroid hypoplasia in all patients. Out of 29 
cases tested for fetal hemoglobin (HbF), 13 cases (44.8%) had high level of HbF. 
Erythroid colony-forming unit of bone marrow was tested in 25 patients, among 
them 12 patients (48%) showed normal plasia, 13 (52%) showed hypoplasia. The 
erythropoietin (EPO) levels of 17 patients were elevated. Karyotypes were 
examined in 28 patients, and showed all normal. The treatment was based on 
corticosteroids and Cyclosporine A. Thirty patients had good response to 
corticosteroid therapy, and 10 of them obtained a sustained 
corticosteroid-induced remission. Twenty cases discontinued corticosteroid 
therapy after remission, as a result, 15 cases (75%) relapsed, moreover all the 
relapsed cases still had good response to corticosteroid. Two relapsed patients 
suffered from aplastic anemia, one of them died of therapy failure. Six patients 
were unresponsive to corticosteroid, 1 of which achieved remission with 
cyclosporine A and the others continued to receive regular transfusions. 3 
patients received iron chelation therapy. It is concluded that the clinical 
characteristics, complete blood count, bone marrow smear, HbF level and EPO 
level are useful to make a diagnosis of DBA. Most patients have a good response 
to corticosteroid therapy, but relapse rate is high when drug was discontinued. 
Patients unresponsive to corticosteroid should receive regular transfusions and 
chelation therapy.