Validation of the Molecular International Prognostic Scoring System in patients with myelodysplastic syndromes

Kewan, Tariq; Bahaj, Waled; Durmaz, Arda; Aly, Mai; Ogbue, Olisaemeka; Carraway, Hetty E.; Sekeres, Mikkael A.; Visconte, Valeria; Gurnari, Carmelo; Maciejewski, Jaroslaw P.

doi:10.1182/blood.2022018896

Cited by 20 publications

(11 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…One study by Meggendorfer and colleagues reported significantly shorter overall survival (OS) in patients harboring both alterations compared to MDS-5q patients lacking SF3B1 mutation, while another study by Malcovati et al found no OS difference in a comparable retrospective cohort. 29 Mutations affecting the genes DNMT3A, TET2, and ASXL1 are frequently identified in MDS as first and driver mutations, including MDS-5q cases. 27 DNMT3A mutations occur in roughly 18% of MDS-5q cases, while TET2 mutations were described in nearly 12% of patients.…”

Section: Other Molecular Alterationsmentioning

confidence: 99%

“…Controversial data surround the prognosis of SF3B1 mutations in MDS‐5q patients. One study by Meggendorfer and colleagues reported significantly shorter overall survival (OS) in patients harboring both alterations compared to MDS‐5q patients lacking SF3B1 mutation, while another study by Malcovati et al found no OS difference in a comparable retrospective cohort 29 …”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Myelodysplastic syndromes del(5q): Pathogenesis and its therapeutic implications

Bruzzese,

Martino,

Mendicino

et al. 2024

European J of Haematology

View full text Add to dashboard Cite

Myelodysplastic syndromes (MDS) encompass a heterogeneous set of acquired bone marrow neoplastic disorders characterized by ineffective hematopoiesis within one or more bone marrow lineages. Nearly half of MDS patients carry cytogenetic alterations, with del(5q) being the most prevalent. Since its first description, del(5q) was consistently correlated with a typical clinical phenotype marked by anemia, thrombocytosis, and a low risk of evolving into acute leukemia. Presently, the World Health Organization (WHO) classification of myeloid neoplasms recognizes a specific subtype of MDS known as “myelodysplastic neoplasm with low blast and isolated del(5q)” identified by the sole presence of 5q deletion or in combination with one other abnormality excluding −7/del(7q). Several studies have sought to unravel the biological processes triggered by del(5q) in the development of MDS, revealing the involvement of various genes localized in specific regions of chromosome 5 referred to as common deleted regions (CDR). This intricate biological landscape makes the MDS cells with del(5q) exceptionally sensitive to lenalidomide. Several studies have confirmed the efficacy of lenalidomide in this context. Regrettably, the response to lenalidomide is not conclusive, prompting ongoing research into biological mechanisms that drive patients toward leukemia and strategies to circumvent lenalidomide resistance and disease progression.

show abstract

Section: Other Molecular Alterationsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Myelodysplastic syndromes del(5q): Pathogenesis and its therapeutic implications

Bruzzese,

Martino,

Mendicino

et al. 2024

European J of Haematology

View full text Add to dashboard Cite

show abstract

“…Bernard et al proposed the molecular upgrade of IPSS, namely IPSS-M, which incorporates the aberrations of 31 genes of confirmed independent impact on MDS prognosis [ 62 ]. The prognostic advantage of IPSS-M has now been validated and challenged over IPSS or IPSS-R in external cohorts [ 63 ]. Similarly, the 2022 European Leukemia Net (ELN) recommendations incorporated mutations in BCOR , EZH2 , SF3B1 , SRSF2 , STAG2 , U2AF1 , and ZRSR2 into the adverse risk category of AML on top of their 2017’s recommendations to define cases with an MDS-like signature [ 64 , 65 ].…”

Section: Hma Resistant Mds/aml Assessment and Prognostication Toolsmentioning

confidence: 99%

What’s Next after Hypomethylating Agents Failure in Myeloid Neoplasms? A Rational Approach

Awada

Gurnari

Xie

et al. 2023

Cancers

Self Cite

View full text Add to dashboard Cite

Hypomethylating agents (HMA) such as azacitidine and decitabine are a mainstay in the current management of patients with myelodysplastic syndromes/neoplasms (MDS) and acute myeloid leukemia (AML) as either single agents or in multidrug combinations. Resistance to HMA is not uncommon, and it can result due to several tumor cellular adaptations. Several clinical and genomic factors have been identified as predictors of HMA resistance. However, the management of MDS/AML patients after the failure of HMA remains challenging in the absence of standardized guidelines. Indeed, this is an area of active research with several potential therapeutic agents currently under development, some of which have demonstrated therapeutic potential in early clinical trials, especially in cases with particular mutational characteristics. Here, we review the latest findings and give a rational approach for such a challenging scenario.

show abstract

“…Studies have consistently shown that IPSS-M surpasses IPSS-R in prognostic accuracy for various clinical outcomes. [7][8][9] In this study, we evaluated the prognostic performances of IPSS-R and IPSS-M for patients with MDS undergoing allogeneic peripheral blood stem cell transplantation (allo-PBSCT) and investigated whether prophylactic intervention could improve the post-transplant survival based on IPSS-M risk category.…”

Section: Introductionmentioning

confidence: 99%

Prophylactic Intervention for Relapse after Allogeneic Peripheral Blood Stem Cell Transplantation Improve the Survival of adult patients with Myelodysplastic Syndrome based on IPSS-M stratification

Song,

Huixian,

Shuang

et al. 2024

Preprint

View full text Add to dashboard Cite

Allogeneic hematopoietic stem cell transplantation represents a curative modality for patients with myelodysplastic syndrome (MDS), yet relapse risk persists. The prognostic performances of the Revised International Prognostic Scoring System (IPSS-R) and the Molecular International Prognostic Scoring System (IPSS-M) for 129 MDS patients undergoing allogeneic peripheral blood stem cell transplantation (allo-PBSCT) were evaluated. Using IPSS-M, 29.5% of patients were reclassified to a higher risk and 14.0% to a lower risk compared to IPSS-R. The two-year recurrence-free survival (RFS) and overall survival (OS) post-transplant were similar across IPSS-R groups, but significantly lower in the very-high risk category with IPSS-M (P=0.005 for RFS; P=0.014 for OS). Multivariate analysis revealed that patient age (P=0.008), very-high risk in IPSS-M (P=0.002), Karnofsky Performance Status score (P=0.003), and Grade 3-4 acute graft-versus-host disease (P=0.014) were independent factors for OS and RFS. Only very-high risk category in IPSS-M was the independent factor affecting cumulative incidence of relapse (P=0.003). Prophylactic interventions for relapse significantly decreased the risk of relapse (P=0.003) and increased the survival of patients in the very-high risk category (P=0.002 for OS, P=0.006 for RFS). The study suggested that the IPSS-M system could identify the high relapse risk patients post-transplant who may benefit from early prophylactic interventions.

show abstract

Validation of the Molecular International Prognostic Scoring System in patients with myelodysplastic syndromes

Cited by 20 publications

References 11 publications

Myelodysplastic syndromes del(5q): Pathogenesis and its therapeutic implications

Myelodysplastic syndromes del(5q): Pathogenesis and its therapeutic implications

What’s Next after Hypomethylating Agents Failure in Myeloid Neoplasms? A Rational Approach

Prophylactic Intervention for Relapse after Allogeneic Peripheral Blood Stem Cell Transplantation Improve the Survival of adult patients with Myelodysplastic Syndrome based on IPSS-M stratification

Contact Info

Product

Resources

About