Fast Five Quiz: Low-Risk Myelodysplastic Syndrome

Medscape

10-06-2025

Myelodysplastic syndrome (MDS) is a heterogeneous group of clonal hematopoietic stem cell disorders, each with varying levels of prognosis. Risk stratification is an important component of MDS; however, even lower-risk variants require a nuanced approach to management, and certain factors can further affect patient risk after diagnosis.
What do you know about low-risk MDS? Check your knowledge with this quick quiz.
Among the gene mutations identified that can influence the prognosis in MDS, SF3B1 mutation is strongly associated with favorable clinical outcomes. Other mutations such as such as TP53 , ASXL1 , EZH2 , ETV6 , and RUNX1 typically lead to poorer clinical outcomes. The National Comprehensive Care Network (NCCN) notes that combining analysis of these mutations in MDS with International Prognostic Scoring System (IPSS) can improve risk stratification beyond the strengths of the IPSS alone, which is standard practice in some treatment centers.
Learn more about cytogenic studies for MDS.
Data have shown that hypocellular bone marrow is one of several factors that predict good response to IST in patients with lower-risk MDS. The NCCN specifically recommends IST in select patients, generally those 'aged ≤60 years and with ≤5% marrow blasts, or those with hypocellular marrows, PNH clone positivity, or STAT-3 mutant cytotoxic T-cell clones.'
Other factors associated with predicted benefit from IST include presence of dysplasia, young age (< 60 years), presence of HLA DR15, female sex, absence of ring sideroblasts, presence of trisomy 8, and relatively short duration of transfusion need.
Learn more about hypocellular marrow in MDS.
A study of patients with very low- and low-risk MDS found that mutations in three genes, IDH1 , IDH2 , and NPM1 , are more common among those patients with direct transformation to AML. NPM1 mutations are the most frequently-seen mutation in AML and are found in approximately 30%-35% of cases among adults.
Mutations in ASXL1 , CBL , and TP53 were found to be associated with progression to higher-risk MDS but not with transformation to AML.
Learn more about cytogenetics in MDS.
According to a review published in JAMA , the median survival time for patients with low and very low risk MDS is 5.3 years and 8.8 years, respectively. This is consistent with other recent data. Collected data from the same JAMA review indicated that the median age for MDS diagnosis is approximately 70 years and is more common in males; chemotherapy and radiation therapy exposure are also reported significant risk factors. Further, the IPSS, revised-IPSS, and WHO classification-based prognostic scoring system are the most frequently used scoring systems for MDS risk.
Learn more about MDS risk staging.
Due to longer survival, patients with low-risk MDS might be given multiple red blood cell transfusions as part of their management (which could potentially lead to iron overload). To decrease iron overload, the NCCN recommends consideration of daily iron chelation with subcutaneous deferoxamine or oral deferasirox after > 20-30 transfusions. This approach is especially recommended for patients with lower-risk MDS or those who are potential candidates for transplantation.
Learn more about iron chelation in MDS.
Editor's Note: This article was created using several editorial tools, including generative AI models, as part of the process. Human review and editing of this content were performed prior to publication.