Abstract
Primary myelodysplastic syndromes (MDS), the most common hematologic malignancy to affect the elderly, are clonal disorders of hematopoietic stem cells. MDS is characterized by an increased but ineffective and dysplastic hematopoiesis as well as peripheral cytopenias. The abnormal hematopoietic clone partly gives rise to mature, but functionally and morphologically abnormal blood cells, and, at least in some cases, is capable of both myeloid and lymphoid differentiation ([1] and reviewed in [2]). The term “Myelodysplastic Syndromes” encompasses a heterogenous group of diseases that can form a continuum from relatively indolent clonally derived refractory anemias with or without ringsideroblasts (RARS, RA) or unilineage thrombocytopenias, to clonal multilineage dysplasias (RCMD) and refractory anemia with excess blasts (RAEB), sometimes also termed oligoblastic leukemia (see currently valid WHO-classification of MDS, Table 6.1). Approximately 1/3 of all MDS patients, more so patients with advanced stage MDS such as RAEB-I and RAEB-II, eventually progress to overt acute myeloid leukemia (AML). One might argue, that the terminus “myelodysplasia” was ill-chosen to denote a clonal neoplastic stem cell disorder, as it implicates a non-neoplastic reactive process, but it nevertheless reflects the most prominent cytological and histological findings required for the diagnosis.
WHO-Classification of the Myelodysplastic Syndromes (adapted from [492])
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Pleyer, L., Neureiter, D., Faber, V., Greil, R. (2010). Myelodysplastic Syndromes (MDS). In: Greil, R., Pleyer, L., Faber, V., Neureiter, D. (eds) Chronic Myeloid Neoplasias and Clonal Overlap Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-79892-8_6
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