BishtRef
Articles Journals Publishers Authors Subjects Most Cited New Papers Year Stats Open Access
journal article Jun 08, 2023

Myelodysplastic syndromes: 2023 update on diagnosis, risk‐stratification, and management

Guillermo Garcia‐Manero
American Journal of Hematology Vol. 98 No. 8 pp. 1307-1325 · Wiley
View at Publisher Save 10.1002/ajh.26984
Abstract
AbstractDisease overviewThe myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic therapy.DiagnosisDiagnosis of MDS is based on morphological evidence of dysplasia upon visual examination of a bone marrow aspirate and biopsy. Information obtained from additional studies such as karyotype, flow cytometry, and molecular genetics is usually complementary and may help refine diagnosis. A new WHO classification of MDS was proposed in 2022. Under this classification, MDS is now termed myelodysplastic neoplasms.Risk‐stratificationPrognosis of patients with MDS can be calculated using a number of scoring systems. All these scoring systems include analysis of peripheral cytopenias, percentage of blasts in the bone marrow, and cytogenetic characteristics. The most commonly accepted system is the Revised International Prognostic Scoring System (IPSS‐R). Recently, genomic data has been incorporated resulting in the new IPSS‐M classification.Risk‐adapted therapyTherapy is selected based on risk, transfusion needs, percent of bone marrow blasts, cytogenetic and mutational profiles, comorbidities, potential for allogeneic stem cell transplantation (alloSCT), and prior exposure to hypomethylating agents (HMA). Goals of therapy are different in lower risk patients than in higher risk and in those with HMA failure. In lower risk, the goal is to decrease transfusion needs and transformation to higher risk disease or AML, as well as to improve survival. In higher risk, the goal is to prolong survival. In 2020, two agents were approved in the US for patients with MDS: luspatercept and oral decitabine/cedazuridine. In addition, currently other available therapies include growth factors, lenalidomide, HMAs, intensive chemotherapy, and alloSCT. A number of phase 3 combinations studies have been completed or are ongoing at the time of this report. At the present time there are no approved interventions for patients with progressive or refractory disease particularly after HMA based therapy. In 2021, several reports indicated improved outcomes with alloSCT in MDS as well as early results from clinical trials using targeted intervention.
Topics

No keywords indexed for this article. Browse by subject →

References
180
[9]
Clonal hematopoiesis in human aging and disease

Siddhartha Jaiswal, Benjamin L. Ebert

Science 10.1126/science.aan4673
[11]
Jaiswal S "Clonal hematopoiesis and atherosclerosis" N Engl J Med (2017)
[16]
Bernard E "Molecular international prognostic scoring system for myelodysplastic syndromes" NEJM Evid (2023)
[20]
The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms

Joseph D. Khoury, Eric Solary, Oussama Abla et al.

Leukemia 10.1038/s41375-022-01613-1
[21]
International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data

Daniel A. Arber, Attilio Orazi, Robert P. Hasserjian et al.

Blood 10.1182/blood.2022015850
[26]
Landscape of genetic lesions in 944 patients with myelodysplastic syndromes

T Haferlach, Y Nagata, V Grossmann et al.

Leukemia 10.1038/leu.2013.336
[30]
Steensma DP "What to tell your patient with clonal hematopoiesis and why: insights from 2 specialized clinics" Blood (2020)
[37]
The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia

Daniel A. Arber, Attilio Orazi, Robert Hasserjian et al.

Blood 10.1182/blood-2016-03-643544

Showing 50 of 180 references

Cited By
168
Contrasting impacts of pretransplant cytoreduction and post‐transplant maintenance on outcomes of allogeneic haematopoietic stem cell transplantation in myelodysplastic syndromes: Real‐world multicentre evidence

YiLin Chen, Xiaotong Zhang · 2026

British Journal of Haematology
Metrics
168
Citations
180
References
Details
Published
Jun 08, 2023
Vol/Issue
98(8)
Pages
1307-1325
License
View
Authors
G
Guillermo Garcia‐Manero

Section of MDS, Department of Leukemia University of Texas MD Anderson Cancer Center Houston Texas United States

Funding
National Institutes of Health Award: CA016672
University of Texas MD Anderson Cancer Center
Cite This Article
Guillermo Garcia‐Manero (2023). Myelodysplastic syndromes: 2023 update on diagnosis, risk‐stratification, and management. American Journal of Hematology, 98(8), 1307-1325. https://doi.org/10.1002/ajh.26984
Related

You May Also Like

Hematological findings and complications of COVID‐19

Evangelos Terpos, Ioannis Ntanasis‐Stathopoulos · 2020

1,384 citations

Multiple myeloma: 2020 update on diagnosis, risk‐stratification and management

S. Vincent Rajkumar · 2020

671 citations

TEG and ROTEM: Technology and clinical applications

David Whiting, James A. DiNardo · 2014

588 citations

Iron deficiency across chronic inflammatory conditions: International expert opinion on definition, diagnosis, and management

Maria Domenica Cappellini, Josep Comin‐Colet · 2017

415 citations

The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports

Deirdra R. Terrell, Laura A. Beebe · 2010

355 citations