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Epidemiology, Etiology, and Clinical Presentation of Myelodysplastic Syndromes

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Diagnosis and Management of Myelodysplastic Syndromes

Abstract

Myelodysplastic syndromes (MDS) comprise a heterogenous family of hematopoietic stem disorders with a median age of onset of 71–76 characterized by ineffective hematopoiesis, a propensity to develop acute myeloid leukemia due to clonal evolution, and significantly curtailed overall survival compared with age matched controls. It has undergone many World Health Organization (WHO) classification revisions since the initial French American and British (FAB) defining criteria in 1982 and became reportable to cancer agencies as a neoplasm in 2001. Incidence data from cancer registries range from 3–5 cases/100,000, rise tenfold by the age of 80, and these numbers are likely significant underestimates of true incidence due to under-reporting and under-investigation. MDS is a universal disease, however disease characteristics differ between Eastern and Western countries. While the pathophysiology of MDS is being elucidated, the etiology of 90% of MDS is elusive with only 10% of cases attributable to prior chemo/radiotherapy or environmental exposures. This chapter explores the disease classification, incidence and prevalence, clinical presentation, and discusses known and suspected etiologies.

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  1. Odette Cancer Center, Sunnybrook Health Sciences Center, Toronto, ON, USA

    Rena Buckstein

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  1. Rena Buckstein
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Correspondence to Rena Buckstein .

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  1. Department of Hematology and Medical Oncology, Cleveland Clinic, Cleveland, OH, USA

    Aziz Nazha

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Buckstein, R. (2020). Epidemiology, Etiology, and Clinical Presentation of Myelodysplastic Syndromes. In: Nazha, A. (eds) Diagnosis and Management of Myelodysplastic Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-030-51878-3_1

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