hematology and oncology
Myelodysplastic Syndrome (MDS)
Treatment of MDS
Symptom amelioration and supportive care Chemotherapy Stem cell transplantation In general, treatment is reserved for symptomatic patients. Symptomatic patients usually require chronic blood and platelet Blood Products . These patients subsequently often develop secondary iron overload. Patients with lower-risk myelodysplastic syndrome and serum ferritin level > 1,000 ng/mL (> 1,000 mcg/L) may benefit from Erythrocyte-stimulating agents (ESA) decrease the severity of anemia in 15 to 20% of patients with MDS, particularly in anemic patients who are not dependent on transfusions and have a serum erythropoietin level < 500 mIU/mL (< 500 IU/L). Treatment with both erythrocyte stimulating agents and granulocyte colony-stimulating factor (G-CSF) may increase the erythroid response rate to nearly 40% in refractory anemia with ringed sideroblasts. However, in all forms of MDS, treatment with growth factors (ESA + G-CSF) does not improve survival and/or reduce risk of transformation to AML.Luspatercepthas been successful in increasing the hematocrit in patients with very low– to intermediate-risk MDS with ringed sideroblasts in whom ESA therapy has failed. Drugs used to treat MDS include Azacitidine Decitabine Lenalidomide Azacitidineis a pyrimidine nucleoside analog.Azacitidineprolongs overall survival compared with supportive care and conventional chemotherapy. Median survival in patients with all subgroups of MDS treated withazacitidineis 21 months. Patients should be treated for at least of 4 to 6 cycles and continue for as long as the patient continues to benefit. Decitabineis also a pyrimidine nucleoside analog. It induces remission in as many as 43% of patients with MDS. It is indicated for treatment of patients with MDS of all subtypes. Azacitidineanddecitabineare epigenetic modulators that hypomethylate DNA. Hypermethylation of certain regions of DNA appears to impair tumor suppressor genes and play a role in oncogenesis in MDS. Lenalidomideis an immunomodulator that is effective in reducing red blood cell transfusion requirements in patients with MDS with deletion 5q syndrome. In patients with hypoplastic MDS, immunosuppression withcyclosporinewith or withoutantithymocyte globulin(ATG) has been effective, as evidenced by cell count improvement and decreased need for transfusion. Hematopoietic Stem Cell Transplantation is the only curative treatment for MDS. Allogeneic hematopoietic stem cell transplantation is indicated in younger, medically fit patients generally in intermediate-2 and high risk groups. 