Definition
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal bone marrow disorders characterized by ineffective hematopoiesis due to functional abnormalities of maturing hematopoietic stem cell (HSC). Abnormal HSC actively proliferates, but is unable to undergo the normal differentiation necessary to produce mature circulating elements of the three hematopoietic lineages (i.e., erythroid, granulocytic, and megakaryocytic) and dies in the bone marrow through programmed cell death or apoptosis. This ineffective hematopoiesis manifests clinically with peripheral blood (PB) cytopenia(s) and morphologically with single lineage or multilineage dysplasia.
With disease progression, HSC in MDS acquires multistep alterations, the accumulation of which may eventually cause transformation to acute myeloid leukemia (AML).
Clinical Features
Incidence and Age
The annual incidence of MDS in the general...
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References and Further Reading
Greenberg, P. L., Tuechler, H., Schanz, J., et al. (2012). Revised international prognostic scoring system for myelodysplastic syndromes. Blood, 120(12), 2454–2465.
Hasserjian, R. P., Orazi, A., Brunning, R. D., et al. (2017). Myelodysplastic syndromes Chapter 6. In S. H. Swerdlow et al. (Eds.), WHO classification of tumours of haematopoietic and lymphoid tissues (Revised 4th ed., pp. 97–120). Lyon: IARC.
Malcovati, L., Germing, U., Kuendgen, A., et al. (2007). Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. Journal of Clinical Oncology, 25(23), 3503–3510.
Malcovati, L., Gallì, A., Travaglino, E., et al. (2017). Clinical significance of somatic mutation in unexplained blood cytopenia. Blood, 129(25), 3371–3378.
Margolskee, E., Hasserjian, R. P., Hassane, D., et al. (2017). Myelodysplastic syndrome, unclassifiable (MDS-U) with 1% blasts is a distinct dubgroup of MDS-U with a poor prognosis. American Journal of Clinical Pathology, 148(1), 49–57.
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Sangiorgio, V.F.I., Orazi, A. (2018). Myelodysplastic Syndromes (MDS). In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4695-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4695-1
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