| OBJECTIVE: To investigate the status of anemia and iron overload in patients with β-thalassemia intermediate(TI)in Guangxi and to explor the effect of thalidomide in patients with TI,so as to improve anemia and the quality of life in patients with TI.METHODS: By using the thalassemia management system in the 303 Hospital of PLA,291 patients with TI admitted to 303 Hospital from 2010 to 2017 were included.Records of blood routine and serum ferritin(SF)were collected.A total of 3218 times of hemoglobin(Hb)were obtained and 1249 times of serum ferritin were obtained.The overall distribution of hemoglobin and serum ferritin was investigated,the mean Hb and SF in different years were observed.The dynamic changes of average Hb and SF were observed and the relevant data were analyzed and processed by SPSS.Iron overload of patients with TI is defined as SF would be more than 800 ng/ml.We observed the effect of thalidomide in seven patients with TI requiring blood transfusion were observed.Four of the patients were transfusion-independent,and three patients were transfusion-dependent.For the patients with transfusion-independent : a minor response was defined as an increment in the Hb concentration of(1-2)g/dl;the major response was Hb increment >2g/dl.For the patients with transfusion-dependent : a minor response was definded as a 50% reduction in blood transfusions;the major response was defined as the no need for a blood transfusion.RESULTS: In order to reduce the error,a total of 184 patients with 3 or more blood routine tests were included in the analysis.The average Hb of 184 patients was(7.2 ±1.7)g / dl.One hundred and sixty-six patients with TI(90.5%)with moderate anemia Hb(6.0-9.0)g /dl and severe anemia with Hb(3.0-6.0)g /dl status.Eighty-one patients(44.0%)with the average Hb was less than 7.0g/dl.From 2010 to 2013,the Hb level gradually decreased as the year grew.Hb level increased year by year during 2014-2017,and Hb level with 8 consecutive years of detection values showed an increasing trend with the increasing of age.One hundred and six patients with ferritin were detected for 3 times or more,were included in the analysis : The average SF was(2394.2±1821.8)ng/ml in 91 patients with TI(85.8g / l).The SF level of TI patients with continuous detection value increased gradually with the increasing of year,and the SF level of patients with continuous detection values increased with the increasing of age.A total of 7 patients were treated with thalidomide.For the four patients with transfusion-independent,Hb increased significantly(≥2.0 g/dl)in three and moderately(1.0–2.0g/dl)in one patient.After 3 months of treatment,Hb increased(3.2 ± 1.2)g/dl compared to pretreatment.Among the three patients with transfusion-dependent,transfusion was terminated after one month of treatment in one patient,and the patients transformed from transfusion-dependent to transfusion-independent.From then on,Hb concentration remained at approximately 10g/dl without transfusion.Although the other two patients were not divorced from transfusion-dependent,but the volume decreased >50%,accompanied by an increase in the average Hb.Simultaneously,average Hb concentration increased from 7.1±1.3 g/dl to 8.5 ±0.9 g/dl in case 5,and from 7.2±1.3 g/dl to 8.4 ± 0.9 g/dl in case 7.Changes in Hb composition were analyzed in patients.The increased Hb after treatment was mainly consisted of Hb F,while Hb A showed little change after treatment,suggesting that thalidomide improved anemia mainly by increasing the production of Hb F in patients with TI.Conclusion: The current status of treatment of TI in Guangxi is not satisfactory.Due to the lack of effective blood transfusion and treatment of iron overload,most patients have severe anemia and iron overload.Thalidomide has a highly effective rate and significant improvement of anemia.It is easy to take orally and cheap,which is suitable for the area with backward economy.Further studies of a larger scale are worthful. |
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