International Journal of Hematology

, Volume 73, Issue 4, pp 405–410 | Cite as

Epidemiological Features of Myelodysplastic Syndromes: Results From Regional Cancer Surveys and Hospital-Based Statistics

Progress in hematology


Although myelodysplastic syndromes (MDS) have been increasingly diagnosed in recent years, precise data on their prevalence and incidence are still lacking. Due to difficulties of diagnosis and classification, large-scale population-based studies that are required for obtaining truly representative data on the epidemiology of MDS are currently not available. Our present knowledge of the incidence and other epidemiological characteristics of MDS is based on a few regional studies performed by authors with a long-lasting interest in these hemopathies. Despite certain limitations, these studies have consistently shown that MDS are relatively common hematological malignancies. Their crude incidence varies from 2.1 to 12.6 cases per 100,000 people per year. Among the age group that is mainly affected, people older than 70 years, we are now faced with incidence rates of about 15 to 50 cases per 100,000 people per year. The recent increase in MDS incidence observed in some studies is probably not the result of an actual increase in the number of cases, but reflects improvements in geriatric medical care and diagnosis.

Key words

MDS Incidence Risk factors 


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  1. 1.
    Harris NL, Jaffe ES, Diebold J, et al. World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the clinical advisory committee meeting-Airlie House, Virginia, November 1997.J Clin Oncol. 1999;17:3835–3849.PubMedGoogle Scholar
  2. 2.
    Bennett JM, Catovsky D, Daniel MT. et al. Proposals for the classification of the myelodysplastic syndromes.Br J Haematol. 1982;51:189–199.PubMedGoogle Scholar
  3. 3.
    Gallagher A, Darley RL, Padua RA. The molecular basis of myelodysplastic syndromes.Haematologica. 1997;191–204.Google Scholar
  4. 4.
    Hamilton-Paterson JL. Preleukaemic anaemia.Acta Haematol. 1949;2:309–316.PubMedCrossRefGoogle Scholar
  5. 5.
    Aul C, Bowen DT, Yoshida Y. Pathogenesis, etiology and epidemiology of myelodysplastic syndromes.Haematologica. 1998;83:71–86.PubMedGoogle Scholar
  6. 6.
    Van Leeuwen FE. Risk of acute myelogenous leukaemia and myelodysplasia following cancer treatment.Baillieres Clin Haematol. 1996;9:57–85.PubMedCrossRefGoogle Scholar
  7. 7.
    Cole P, Sateren W, Delzell E. Epidemiologic perspectives on myelodysplastic syndromes and leukemia.Leuk Res. 1995;19:361–365.PubMedCrossRefGoogle Scholar
  8. 8.
    Reizenstein P, Dabrowski L. Increasing prevalence of the myelodysplastic syndrome. An international Delphi study.Anticancer Res. 1991;11:1069–1070.PubMedGoogle Scholar
  9. 9.
    öst A, Reizenstein P. Minimal diagnostic criteria for the myelodys-plastic syndrome.Leuk Res. 1992;16:9–11.PubMedCrossRefGoogle Scholar
  10. 10.
    Foucar K, Langdon RM, Armitage JO, Olson DB, Carroll TJ. Myelodysplastic syndromes. A clinical and pathologic analysis of 109 cases.Cancer. 1985;56:553–561.PubMedCrossRefGoogle Scholar
  11. 11.
    Juneja SK, Imbert M, Jouault H, Scoazec JY, Sigaux F, Sultan C. Haematological features of primary myelodysplastic syndromes (PMDS) at initial presentation: a study of 118 cases.J Clin Pathol. 1983;36:1129–1135.PubMedCrossRefGoogle Scholar
  12. 12.
    Teerenhovi L., Lintula R. Natural course of myelodysplastic syndromes—Helsinki experience.Scand J Haematol. 1986;36(suppl 45):102–106.Google Scholar
  13. 13.
    Cunningham I, MacCallum SJ, Nicholls MD, et al. The myelodys-plastic syndromes: an analysis of prognostic factors in 226 cases from a single institution.Br J Haematol. 1995;90:602–606.PubMedCrossRefGoogle Scholar
  14. 14.
    Kerkhofs H, Hermans J, Haak HL, Leeksma CHW. Utility of the FAB classification for myelodysplastic syndromes: investigation of prognostic factors in 237 cases.Br J Haematol. 1987;65:73–81.PubMedCrossRefGoogle Scholar
  15. 15.
    Sanz GF, Sanz MA, Vallespi T, et al. Two regression models and a scoring system for predicting survival and planning treatment in myelodysplastic syndromes: a multivariate analysis of prognostic factors in 370 patients.Blood. 1989;74:395–408.PubMedGoogle Scholar
  16. 16.
    Morel P, Hebbar M, Lai JL, et al. Cytogenetic analysis has strong independent prognostic value in de novo myelodysplastic syndromes and can be included in a new scoring system: a report on 408 cases.Leukemia. 1993;7:1315–1324.PubMedGoogle Scholar
  17. 17.
    Goasguen JE, Garand R, Bizet M, et al. Prognostic factors of myelodysplastic syndromes—a simplified 3-D scoring system.Leuk Res. 1990;14:255–262.PubMedCrossRefGoogle Scholar
  18. 18.
    Maschek H, Gutzmer R, Choritz H, Georgii A. Life expectancy in primary myelodysplastic syndromes: a prognostic score based upon histopathology from bone marrow biopsies of 569 patients.Eur J Haematol. 1994;53:280–287.PubMedGoogle Scholar
  19. 19.
    Greenberg P, Cox C, LeBeau MM, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes.Blood. 1997;89:2079–2088.PubMedGoogle Scholar
  20. 20.
    Oguma S, Yoshida Y, Uchino H, Maekawa T, Nomura T, Mizoguchi H. Clinical characteristics of Japanese patients with primary myelodysplastic syndromes: a co-operative study based on 838 cases.Leukemia. 1995;19:219–225.CrossRefGoogle Scholar
  21. 21.
    Paydas S, Kocak R. Younger age observation in myelodysplastic syndrome [letter].Leuk Res. 1996;20:367.PubMedCrossRefGoogle Scholar
  22. 22.
    Fenaux P, Preudhomme C, Estienne MH, et al. De novo myelodys-plastic syndromes in adults aged 50 or less. A report on 37 cases.Leuk Res. 1990;14:1053–1059.PubMedCrossRefGoogle Scholar
  23. 23.
    Ribera JM, Cervantes F, Rozman C. A multivariate analysis of prognostic factors in chronic myelomonocytic leukaemia according to the FAB criteria.Br J Haematol. 1987;65:307–311.PubMedCrossRefGoogle Scholar
  24. 24.
    Van den Berghe H, Vermaelen K, Mecucci C, Barbieri D, Tricot G. The 5q-anomaly.Cancer Genet Cytogenet. 1985;17:189–225.PubMedCrossRefGoogle Scholar
  25. 25.
    Pedersen B. 5q- survival: importance of gender and deleted 5q bands and survival analysis based on 324 published cases.Leuk Lymphoma. 1998;31:325–334.PubMedGoogle Scholar
  26. 26.
    Aul C, Gattermann N, Schneider W. Age-related incidence and other epidemiological aspects of myelodysplastic syndromes.Br J Haematol. 1992;82:358–367.PubMedCrossRefGoogle Scholar
  27. 27.
    Barrett J, Saunthararajah Y, Molldrem J. Myelodysplastic syndromes and aplastic anemia: distinct entities or diseases linked by a common pathophysiology?Semin Hematol. 2000;37:15–29.PubMedCrossRefGoogle Scholar
  28. 28.
    Williamson PJ, Kruger AR, Reynolds PJ, Hamblin TJ, Oscier DG. Establishing the incidence of myelodysplastic syndrome.Br J Haematol. 1994;87:743–745.PubMedCrossRefGoogle Scholar
  29. 29.
    Cartwright RA. Incidence and epidemiology of the myelodys-plastic syndromes. In: Mufti GJ, Galton DAG, eds. The Myelodys-plastic Syndromes. Edinburgh, Scotland: Churchill Livingstone. 1992:23–31.Google Scholar
  30. 30.
    Radlund A, Thiede T, Hansen S, Carlsson M, Engquist L. Incidence of myelodysplastic syndromes in a Swedish population.Eur J Haematol. 1995;54:153–156.PubMedCrossRefGoogle Scholar
  31. 31.
    Maynadié M, Verret C, Moskovtchenko P, et al. Epidemiological characteristics of myelodysplastic syndromes in a well-defined French population.Br J Cancer. 1996;74:288–290.PubMedGoogle Scholar
  32. 32.
    Bauduer F, Ducout L, Dastugue N, Capdupuy C, Renoux M. Epidemiology of myelodysplastic syndromes in a French general hospital of the Basque Country.Leuk Res. 1998;22:205–208.PubMedCrossRefGoogle Scholar
  33. 33.
    Shimizu H, Matsushita Y, Aoki K, Nomura T, Yoshida Y, Mizoguchi H. Prevalence of the myelodysplastic syndromes in Japan.Int J Hematol. 1995;61:17–22.PubMedCrossRefGoogle Scholar
  34. 34.
    Saarni MI, Linman JW. Preleukemia: the hematologic syndrome preceding acute leukemia.Am J Med. 1973;55:38–48.PubMedCrossRefGoogle Scholar
  35. 35.
    McNally RJQ, Roman E, Cartwright RA. Leukemias and lym-phomas: time trends in the UK, 1984-93.Cancer Causes Control. 1999;10:35–42.PubMedCrossRefGoogle Scholar
  36. 36.
    Aul C, Gattermann N, Germing U, Runde V, Heyll A. Myelodys-plastische Syndrome. Epidemiologische und ätiologische Aspekte.Dtsch Med Wschr. 1992;117:1223–1231.PubMedGoogle Scholar
  37. 37.
    Pedersen-Bjergaard J, Philip P, Larsen SO, et al. Therapy-related myelodysplasia and acute myeloid leukemia. Cytogenetic characteristics of 115 consecutive cases and risk in seven cohorts of patients treated intensively for malignant diseases in the Copenhagen series.Leukemia. 1993;7:1975–1986.PubMedGoogle Scholar
  38. 38.
    Pui CH, Ribeiro RC, Hancock ML, et al. Acute myeloid leukemia in children treated with epipodophyllotoxins for acute lym-phoblastic leukemia.N Engl J Med. 1991;325:1682–1687.PubMedGoogle Scholar
  39. 39.
    Travis LB, Li CY, Zhang ZN, et al. Hematopoietic malignancies and related disorders among benzene-exposed workers in China.Leuk Lymphoma. 1994;14:91–102.PubMedCrossRefGoogle Scholar
  40. 40.
    Honda Y, Delzell E, Cole P. An updated study of mortality among workers at a petroleum manufacturing plant.J Occup Envir Med. 1995;37:194–200.CrossRefGoogle Scholar
  41. 41.
    Nisse C, Lorthois C, Dorp V, Eloy E, Haguenoer JM, Fenaux P. Exposure to occupational and environmental factors in myelodys-plastic syndromes. Preliminary results of a case-control study.Leukemia. 1995;9:693–399.PubMedGoogle Scholar
  42. 42.
    Mele A, Szklo M, Visani G, et al. Hair dye use and other risk factors for leukemia and pre-leukemia: a case-control study.Am J Epidemiol. 1994;139:609–619.PubMedGoogle Scholar
  43. 43.
    Correa A, Mohan A, Jackson L, Perry H, Helzlsouer K. Use of hair dyes, hematopoietic neoplasms, and lymphomas: a literature review, I: leukemias and myelodysplastic syndromes.Cancer Invest. 2000;18:366–380.PubMedCrossRefGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2001

Authors and Affiliations

  1. 1.Medizinische Klinik II (Hämatologie, Onkologie, und Immunologie)St. Johannes-Hospital DuisburgDuisburgGermany

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