Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Myelodysplastic Syndromes (MDS)

  • Valentina F. I. Sangiorgio
  • Attilio Orazi
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4695-1

Synonyms

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...

This is a preview of subscription content, log in to check access.

References and Further Reading

  1. Greenberg, P. L., Tuechler, H., Schanz, J., et al. (2012). Revised international prognostic scoring system for myelodysplastic syndromes. Blood, 120(12), 2454–2465.CrossRefPubMedCentralPubMedGoogle Scholar
  2. 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.Google Scholar
  3. 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.CrossRefPubMedGoogle Scholar
  4. Malcovati, L., Gallì, A., Travaglino, E., et al. (2017). Clinical significance of somatic mutation in unexplained blood cytopenia. Blood, 129(25), 3371–3378.PubMedCentralPubMedGoogle Scholar
  5. 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.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory MedicineWeill Cornell Medical CollegeNew YorkUSA
  2. 2.Universita’ degli Studi di MilanoMilanItaly