Myelodysplastic syndromes/neoplasms (MDS) are a heterogeneous group of hematopoietic neoplasms characterized by ineffective hematopoiesis, cytopenia, and a variable risk of progression to acute myeloid leukemia (AML) (1, 2). The classical hallmark of MDS is the presence of dysplastic changes in one or more myeloid lineages associated with ineffective maturation of hematopoietic precursors responsible for peripheral cytopenias. Therefore, the clinical picture of MDS encompasses signs and symptoms of anemia, bleeding, and recurrent infections(3, 4). Risk stratification in MDS is crucial to guide the treatment goals and different therapeutic strategies(4, 5). In addition, precise risk assessment is essential for estimation of outcomes, including overall survival (OS) and risk of progression to AML (6). The recent advancement in next generation sequencing (NGS) allowed for a better understanding of MDS pathogenesis and led to the discovery of multiple molecular alterations (7, 8, 9). Some genetic mutations and cytogenetic abnormalities have been described and correlated with different morphological and phenotypic subtypes of MDS(2, 9). Such molecular alterations are related to different aspects of MDS pathogenesis, risk stratification, and response to specific treatments(10).

Multiple prognostic tools and scoring systems have been established over the years to help guide MDS management and risk stratification (11). The International Prognostic Scoring System (IPSS), revised International Prognostic Scoring System (IPSS-R), MD Anderson prognostic scoring system, and WHO classification-based Prognostic scoring system (WPSS), incorporated clinical and laboratory data at the time of diagnosis to assign patients into different risk groups correlated with outcomes(12, 13, 14, 15). Although widely validated, several limitations have been reported using the traditional clinical scores such as heterogeneity in prognosis among patients assigned to a similar risk group, questionable dynamicity, and the absence of molecular mutations and their effects on the overall MDS prognosis and treatment response, among others. Based on that, many efforts and studies started to incorporate molecular data to develop molecular scoring systems to cope with the advancement in NGS and improve risk stratification, treatment selections, and clinical trial assignments(16, 17, 18). In the current review, we will summarize the available MDS molecular scoring system and highlight their feasibility, additive value and limitations compared to the traditional clinical scoring systems.