Diagnosis and Treatment of Low-Risk Myelodysplastic Syndrome / 实用儿科临床杂志

ABSTRACT

Objective To study the clinical features of diagnosis and treatment in children with low-risk myelodysplastic syndrome(MDS) and improve the diagnosis and treatment.Methods The examinations of 17 children with low-risk MDS were analyzed.The blood concentrations of cyclosporine,one of the therapeutic drugs,were monitored and the responses to the treatments were evaluated.Results Exa-mination of full blood count showed that the reductions of 3 cell types,2 cell types and 1 cell type were 11 (64.7%)cases,5(29.4%)cases and 1(5.9%)case,respectively.Reticulocyte count showed an increase in 82.4% of the patients and normal in 3 cases.Fourteen in 17 cases were hyperplastic marrow and 3 cases were hypoplastic marrow.Among all cases,one lineage,2 lineages and 3 lineages dyspoiesis were seen in 8(47.1%),7(41.2%) and 1 (5.9%)cases,respectively.One case showed no dyspoiesis.Cytogenetics examination showed normal in 10(58.8%) cases and abnormal in 7(41.2%) cases.Fifteen (88.2%) cases had normal proportions of CD59 negative cells,while 2 cases had higher proportions.The blood concentrations of cyclosporine that were tested in 9 cases at the end of the third week were in a range of 95.3-316.5 ?g/L.The therapeutic effect of 10 cases were evaluated at the end of the third month after being treated.Eight cases achieved haematological improvement and 2 cases didn′t.The rate of improvement was 80%.Conclusions The patients of low-risk MDS are mostly school-aged children and pancytopenia is the most common sign.The combination of predisone,cyclosporine and stanozolol agents shows good effect to treat low-risk MDS.The absorption of cyclosporine is different individually,so it is significant to adjust the dosage of cyclosporine according to the concentration regularly in clinical practice.