Myelodysplastic syndrome from theoretical review to clinical application view

Abstract

Myelodysplastic syndromes (MDS), called ineffective hematopoiesis is indicated by bone marrow failure and tendency to acute myeloid leukemia transformation. Since the disease is more common in elderly with non- hematology co-morbidities, the research for less toxic and curative novel agents is essential. More than 12 years without new Food and Drug Administration approved drugs in MDS management through the whole course, only 5 drugs. We summarized the basic data in diagnosis, treatment guidelines and future direction.

Keywords: Myelodysplastic syndrome; hypomethyating agents; lenalidomide.

Figure 1.

A) Proposed algorithm for low risk (IPSS low, intermediate; IPSS-R or WPSS, very low, low or intermediate) management. Chromosomal abnormality and with symptomatic anemia follow the same algorithm (except chromosome 7). a) The lenalidomide standard dose is 10 mg/day for 3 weeks and 1 week of (3/4), however, in the presence of significant neutropenia (platelet count <25000 cell/mcl) ± neutrophil count <500 cell/mcl), the dose may be modified or withdrawal; b) ESAs included darbepoetin alfa (150-300 mcg SC every other week) and human recombinant Epo (40,000 to 60,000 SC units twice/week); c) Ring sideroblasts .15% in BM and EPO.500 mU/Ml are predictive markers for better response. 1-2mcg/kg twice/week in the standard dose of G-CSF; d) Treatment failure is defined as loss of the response after 3 months. We must exclude iron deficiency. Response evaluation according to IWG; e) Criteria for IST are HLA-DR15 histocompatibility type, low risk, normal cytogenetics, hypocellular BM, and PNH clone. B) Proposed algorithm for high risk MDS (IPSS int-2, high; IPSS-R intermediate, high, very high; WPSS high, very high) management. IST: immunosuppressive treatment, HCT: Hematopoietic stem cell transplantation.