History and Epidemiology of Myelodysplastic Syndromes

Haema 2011; 2(2): 136-143

by Maria Papaioannou, Sofia Mpampali, George Karamarias

1st Department of Internal Medicine, Hematology Dept, Medical School, Aristotle University of Thessaloniki, AHEPA Hospital, Thessaloniki, Greece

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The conditions that currently come under the term Myelodysplastic Syndromes (MDS) have been recognised for over a century under a variety of names. The first classification of the MDS published in 1982 by the French-American-British (FAB) group, which defined sideroblastic anemia and included chronic myelomonocytic leukemia. In 1997, Greenberg et al, published the International Prognostic Scoring System (IPSS) which has been extremely useful for many years. In 2000 the initial WHO classification was developed and revised in 2008. Sideroblastic anemia (WHO RARS) first introduced in 1956 by Bjorkman and the 5q- syndrome (WHO refractory anemia with an isolated del(5q) described by Van Den Berghe in 1974, are two entities with clearly different biological basis which are considered as MDS by WHO. Myelodysplastic syndromes (MDS) are one of the most common hematological disorders among the elderly. Approximately 80% of the patients are older than 60 years at diagnosis. The true incidence of MDS is largely unknown. There is a lack of reliable data concerning the epidemiology and etiology of MDS due to difficulties in diagnosis and classification, which have made large-scale population-based studies problematic. Several studies have estimated an incidence, ranging from 1 to 12.6 cases per 100,000 people per year. Undoubtedly, the risk of MDS development increases with aging. Etiological factors of the (MDS) are basically unknown, with the exception of alkylating agents, ionizing radiation and benzene. Nowadays, a variety of risk factors have been linked with increased risk (smoking and exposure to solvents and agrochemicals) or decreased risk of MDS (wine drinking), but the majority of cases remain unexplained. Much still needs to be learned about what causes MDS and the genetic factors that increase susceptibility.