AI Article Synopsis
- Myelodysplastic syndromes (MDS) are a group of diverse blood disorders characterized by ineffective blood cell production and varying clinical outcomes, classified into 5 risk groups by the revised international prognostic scoring system (IPSS-R).
- Treatment for lower-risk patients typically involves supportive care with red blood cell transfusions, iron chelation, and medications like erythropoiesis-stimulating agents and lenalidomide for specific cases.
- Higher-risk patients may require allogeneic stem cell transplantation, with alternative options like hypomethylating agents if transplantation isn't feasible, while new therapies and combinations are being researched to improve outcomes for both lower- and higher-risk patients.
Article Abstract
: Myelodysplastic syndromes (MDS) comprise a heterogeneous group of myeloid neoplasms with diverse clinical courses. The revised version of the international prognostic scoring system (IPSS-R) provides risk stratification into 5 different groups. : For lower-risk patients, red blood cell transfusions and iron chelation are the backbone of supportive care. In addition, erythropoiesis-stimulating agents (ESA) are used to ameliorate anemia. Lenalidomide is approved for the treatment of lower-risk patients with del(5q) who are transfusion-dependent. Patients with higher-risk disease should be offered allogeneic stem cell transplantation whenever possible. If they are unfit for transplantation or an appropriate donor cannot be found, hypomethylating agents may be used. : New therapeutic options for lower-risk patients include thrombopoietin analogues, the TGF-beta family ligand trapping drug Luspatercept, and the telomerase inhibitor Imetelstat. Combinations of hypomethylating agents (HMA) with other compounds, and inhibitors of bcl2, such as venetoclax are being developed for higher-risk patients. Finally, hypomethylating agents in combination with donor lymphocytes may lead to long-term remission following molecular or hematological relapse after allogeneic SCT.
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| http://dx.doi.org/10.1080/17474086.2019.1647778 | DOI Listing |
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