4.2 Review

Myelodysplastic Syndromes: A New Decade

Journal

CLINICAL LYMPHOMA MYELOMA & LEUKEMIA
Volume 22, Issue 1, Pages 1-16

Publisher

CIG MEDIA GROUP, LP
DOI: 10.1016/j.clml.2021.07.031

Keywords

Hypomethylating agents; IPSS-R; High risk; Low risk; Novel therapies; MDS; Somatic muations

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Myelodysplastic syndromes (MDS) is a group of heterogeneous clonal hematopoietic stem cell disorders with an increasing incidence rate with age. Risk stratification, prognosis, survival, and AML transformation in MDS depend largely on the revised International Prognostic Scoring System and molecular genetic testing. Treatment varies based on disease severity, with low-risk MDS aimed at alleviating anemia symptoms and high-risk MDS requiring prompt initiation of hypomethylating agents. Allogeneic stem cell transplant may be the only potential cure for high-risk MDS.
Myelodysplastic syndromes (MDS) are a group of heterogeneous clonal hematopoietic stem cell disorders. The 2020 Surveillance, Epidemiology, and End Results data demonstrates the incidence rate of MDS increases with age especially in those greater than 70 years of age. Risk stratification that impact prognosis, survival, and rate of acute myeloid leukemia (AML) transformation in MDS is largely dependent on revised International Prognostic Scoring System along with molecular genetic testing as a supplement. Low risk MDS typically have a more indolent disease course in which treatment is only initiated to ameliorate symptoms of cytopenias. In many, anemia is the most common cytopenia requiring treatment and erythroid stimulating agents, are considered first line. In contrast, high risk MDS tend to behave more aggressively for which treatment should be initiated rapidly with Hypomethylating Agents (HMA) being in the frontline. In those with high risk MDS and eligible, evaluation for allogeneic stem cell transplant should be considered as this is the only potential curative option for MDS. With the use of molecular genetic testing, a personalized approach to therapy in MDS has ensued. As the treatment landscape in MDS continues to flourish with novel targeted agents, we ambitiously seek to improve survival rates especially among the relapsed/refractory and transplant ineligible. (C) 2021 Elsevier Inc. All rights reserved.

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