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Welcome to the The Myelodysplastic Syndromes (MDS) Channel
Explore all things new and exciting in MDS, including expert interviews from major international congresses, feature articles and webinars.
MDS is a bone marrow failure disorder whereby blast cell maturation is hindered, causing the accumulation of dysplastic cells within the bone marrow and rendering the differentiation process suboptimal.
Paradoxically, the hypercellular bone marrow results in ineffective hematopoiesis and thus the hallmark MDS cytopenia’s. Subsequently, infection, anemia, spontaneous bleeding, and petechiae are all typical of this malignancy.1
Based on the Revised International Prognostic Scoring System (IPSS-R), which takes into account bone marrow cytogenetics, marrow blast percentage, and cytopenias, patients are divided into categories.2 Ranging from very low-risk to very high-risk, patients typically have a median survival of 8.8 to 0.8 years, respectively.
Moreover, around 30% of patients will progress to acute myeloid leukemia (AML).3Known to be a disease of the elderly, it is estimated that 10,000 new cases of MDS arise in the US per annum.4 Additionally, with an aging population, increased rates of secondary MDS, and a greater awareness of the disease, diagnosis rates will likely accelerate.
As with many hematological cancers, the etiology of MDS is predominantly idiopathic, but risk-factors have been identified. These include exposure to ionizing radiation and chemotherapy agents, as well as certain chemicals, such as Agent Orange and hydrocarbons. Additionally, congenital conditions such as Fanconi anemia can also increase risk.1
With hematopoietic stem cell transplantation (HSCT) representing the only curative option, MDS has not seen the rapid evolution in treatments witnessed in other hematological malignancies. Many patients receive only remedies for their cytopenias, such as erythropoiesis-stimulating agents and transfusions, whereas higher-risk patients receive hypomethylating agents and/or HSCT.
Despite the limited treatment options, the field is researching targets for new therapies and is increasingly utilizing personalized medicine. Immunotherapies such as the monoclonal antibody magrolimab are currently being researched as are first-in-class agents like the TCA cycle targeting devimistat and pevonedistat, an inhibitor of NEDD8 activating enzyme (NAE).
- Fazal S. (2019). ‘Therapies and Treatment Options 2019’ [PowerPoint presentation]. Available at: https://www.mds-foundation.org/wp-content/uploads/2019/02/MDS-Foundation-2019-Final.pdf (Last Accessed: 11/03/20).
- Greenberg P, Tuechler H, Schanz J et al. Revised International Prognostic Scoring System for Myelodysplastic Syndromes. 2012 Sept;120(12): 2454–2465.
- MDS-Foundation.org [Internet]. What is MDS? Available from: https://www.mds-foundation.org/what-is-mds/ (Last Accessed: 11/03/20).