HJNO Jan/Feb 2021

52 JAN / FEB 2021 I  HEALTHCARE JOURNAL OF NEW ORLEANS ONCOLOGY • Risk stratification by IPSS; • Prognostic relevance of somatic and germline mutation. Treatment • Active surveillance and deferred treat- ment – for patients with mild and as- ymptomatic MDS; • Transfusion at about 8gms – preferred, but often patients may feel better with earlier intervention. • Erythropoietic stimulating agents – if erythropoietin level is less than 200, it has best chance for a response.; • Luspatercept in MDS with RARS can lead to transfusion independence for 8% of patients (role in all risk MDS is being explored); • Immunosuppressive agents – for hypo- plastic marrow for young patients who are not transplant candidates; • Iron chelation and transfusion with des- ferrioxamine for iron overload; • Hypomethylating agents for moderate to high-risk patients; • Azacitidine; • Decitabine occ. combinations with lenalidomide; • Venetoclax seems to prolong survival in the high risk but is still not the stan- dard of care; • Lenalidomide can induce lasting remis- sion in 5q- and other syndromes; • Intensive chemotherapy for patients below 60 years of age with AML doses or CPX-351, a liposomal combination of daunorubicin and ara-c; • Novel targeted agents against TP53, IDH2 and IDH1 is present in about 10% of patients. Enasidenib of IDH muta- tions and APR-246 for TP53 are on- going. CC-486 oral azacitidine shows early promise in low risk, as well as Im- etelstat, a telomerase inhibitor. Anti-CD 47 showing 91% response in a very small study; • Stem cell transplant – for the high grade and germline mutations and in hypo- plastic MDS. Conclusions MDS/MPN are complex malignant multi- faceted diseases. New categories are “coined” as we decipher their biological defects, which then become treatment targets! Research in MDS has intensified as our population ages and with a large population of smokers and cancers survivors. Unfortunately, cure is still achieved in only a few. While morbidity and mortality continue to improve, challenges remain. This article cannot do full justice in this rapidly evolving disease, though new ther- apeutic options are available literally on a monthly basis. So, stay tuned! n Jitendra G. Gandhi, MD, earned a medical degree at Seth G.S. Medical College, Bombay University in Bombay, India. He completed an internal medicine residency at Worcester City Hospital, in Worcester, Massachusetts,where he served as chief resident.He then went on to complete a fellowship in hematology/ oncology at the University of Massachusetts Medical Center. He is board certified in internal medicine and medical oncology. Gandhi has extensive oncology expertise participating in numerous clinical research studies as a principle investigator with various types of cancers. A peripheral blood smear in myelofibrosis. Grossly deformed and bizarre RBC’s.