Skip to main content

Advertisement

SpringerLink
Log in

Myelodysplastic syndrome: An update on diagnosis and therapy

  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

Myelodysplastic syndromes (MDS) are a diverse group of disorders characterized by disorderly and ineffective hematopoiesis. Patients suffer morbidity from associated cytopenias that result in an increased risk of infection, transfusion-dependent anemia, and bleeding. Despite the variable risk of transformation to acute leukemia, the majority of deaths are due to bone marrow failure. No truly effective treatment exists for MDS, and therapy usually focuses on reducing or preventing complications of the disease. Identification of potential cellular and molecular targets, such as epigenetic modification, has led to novel therapeutic approaches in recent years. An increasing number of diagnostic markers, prognostic parameters, and therapeutic strategies are available and becoming widely accepted.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Bennett JM, Catovsky D, Daniel MT: Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 1982, 51:189–199.

    PubMed  CAS  Google Scholar 

  2. 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.

    PubMed  CAS  Google Scholar 

  3. Aul C, Giagounidis A, Germing U: Epidemiological features of myelodysplastic syndromes: results from regional cancer surveys and hospital-based statistics. Int J Hematol 2001, 73:405–410.

    Article  PubMed  CAS  Google Scholar 

  4. Mathew P, Tefferi A, Dewald GW, et al.: The 5q-syndrome: a single-institution study of 43 consecutive patients. Blood 1993, 81:1040–1045.

    PubMed  CAS  Google Scholar 

  5. Greenberg P, Cox C, LeBeau MM, et al.: International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997, 89:2079–2088. Erratum in: Blood 1998, 91:1100.

    PubMed  CAS  Google Scholar 

  6. Billström R, Johansson H, Johansson B, Mitelman F: Immune-mediated complications in patients with myelodysplastic syndromes: clinical and cytogenetic features. Eur J Haematol 1995, 55:42–48.

    Article  PubMed  Google Scholar 

  7. Chandran G, Ahern MJ, Seshadri P, Coghlan D: Rheumatic manifestations of the myelodysplastic syndromes: a comparative study. Aust N Z J Med 1996, 26:683–688.

    PubMed  CAS  Google Scholar 

  8. Enright H, Jacob HS, Vercellotti G, et al.: Paraneoplastic autoimmune phenomena in patients with myelodysplastic syndromes: response to immunosuppressive therapy. Br J Haematol 1995, 91:403–408.

    Article  PubMed  CAS  Google Scholar 

  9. Saif MW, Hopkins JL, Gore SD: Autoimmune phenomena in patients with myelodysplastic syndromes and chronic myelomonocytic leukemia. Leuk Lymphoma 2002, 43:2083–2092.

    Article  PubMed  Google Scholar 

  10. Tefferi A, Hoagland HC, Therneau TM, Pierre RV: Chronic myelomonocytic leukemia: natural history and prognostic determinants. Mayo Clin Proc 1989, 64:1246–1254.

    PubMed  CAS  Google Scholar 

  11. Soppi E, Nousiainen T, Seppa A, Lahtinen R: Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in association with myelodysplastic syndromes: a report of three cases and a review of the literature. Br J Haematol 1989, 73:43–47.

    Article  PubMed  CAS  Google Scholar 

  12. French registry of acute leukemia and myelodysplastic syndromes. Age distribution and hemogram analysis of the 4496 cases recorded during 1982–1983 and classified according to FAB criteria. Groupe Francais de Morphologie Hematologique. Cancer 1987, 60:1385–1394.

  13. Bowen D, Culligan D, Jowitt S, et al.: Guidelines for the diagnosis and therapy of adult myelodysplastic syndromes. Br J Haematol 2003, 120:187–200.

    Article  PubMed  Google Scholar 

  14. Michels SD, McKenna RW, Arthur DC, et al.: Therapy-related acute myeloid leukemia and myelodysplastic syndrome: a clinical and morphologic study of 65 cases. Blood 1985, 65:1364–1372.

    PubMed  CAS  Google Scholar 

  15. Horny HP, Sotlar K, Valent P: Diagnostic value of histology and immunohistochemistry in myelodysplastic syndromes. Leuk Res 2007, 31:1609–1616.

    Article  PubMed  CAS  Google Scholar 

  16. Tricot G, De Wolf-Peeters C, Vlietinck R, Verwilghen RL: Bone marrow histology in myelodysplastic syndromes. II. Prognostic value of abnormal localization of immature precursors in MDS. Br J Haematol 1984, 58:217–225.

    Article  PubMed  CAS  Google Scholar 

  17. Oriani A, Annaloro C, Soligo D, et al.: Bone marrow histology and CD34 immunostaining in the prognostic evaluation of primary myelodysplastic syndromes. Br J Haematol 1996, 92:360–364.

    Article  PubMed  CAS  Google Scholar 

  18. Panani AD, Roussos C: Cytogenetic aspects of adult primary myelodysplastic syndromes: clinical implications. Cancer Lett 2006, 235:177–190.

    Article  PubMed  CAS  Google Scholar 

  19. Bernasconi P, Alessandrino EP, Boni M, et al.: Karyotype in myelodysplastic syndromes: relations to morphology, clinical evolution, and survival. Am J Hematol 1994, 46:270–277.

    Article  PubMed  CAS  Google Scholar 

  20. de Souza Fernandez T, Ornellas MH, Otero de Carvalho L, et al.: Chromosomal alterations associated with evolution from myelodysplastic syndrome to acute myeloid leukemia. Leuk Res 2000, 24:839–848.

    Article  PubMed  Google Scholar 

  21. Valent P, Horny HP, Bennett JM, et al.: Definitions and standards in the diagnosis and treatment of the myelodysplastic syndromes: Consensus statements and report from a working conference. Leuk Res 2007, 31:727–736.

    Article  PubMed  Google Scholar 

  22. Haase D, Germing U, Schanz J, et al.: New insights into the prognostic impact of the karyotype in MDS and correlation with subtypes: evidence from a core dataset of 2124 patients. Blood 2007, 110:4385–4395.

    Article  PubMed  CAS  Google Scholar 

  23. Garcia-Manero G: Modifying the epigenome as a therapeutic strategy in myelodysplasia. Hematology Am Soc Hematol Educ Program 2007, 405–411.

  24. Herman JG, Baylin SB: Gene silencing in cancer in association with promoter hypermethylation. N Engl J Med 2003, 49:2042–2054.

    Article  Google Scholar 

  25. Lubbert M: DNA methylation inhibitors in the treatment of leukemias, myelodysplastic syndromes and hemoglobinopathies: clinical results and possible mechanisms of action. Curr Top Microbiol Immunol 2000, 249:135–164.

    PubMed  CAS  Google Scholar 

  26. Daskalakis M, Nguyen TT, Nguyen C, et al.: Demethylation of a hypermethylated P15/INK4B gene in patients with myelodysplastic syndrome by 5-aza-2′-deoxycytidine (decitabine) treatment. Blood 2002, 100:2957–2964.

    Article  PubMed  CAS  Google Scholar 

  27. Uchida T, Kinoshita T, Nagai H, et al.: Hypermethylation of the p15/INK4B gene in myelodysplastic syndromes. Blood 1997, 4:1403–1409.

    Google Scholar 

  28. Quesnel B, Guillerm G, Vereecque R, et al.: Methylation of the p15/INK4B gene in myelodysplastic syndromes is frequent and acquired during disease progression. Blood 1998, 8:2985–2990.

    Google Scholar 

  29. Ebert BL, Pretz J, Bosco J, et al.: Identification of RPS14 as a 5q-syndrome gene by RNA interference screen. Nature 2008, 451:335–339.

    Article  PubMed  CAS  Google Scholar 

  30. Mohamedali A, Gäken J, Twine NA, et al.: Prevalence and prognostic significance of allelic imbalance by single-nucleotide polymorphism analysis in low-risk myelodysplastic syndromes. Blood 2007, 110:3365–3373.

    Article  PubMed  CAS  Google Scholar 

  31. Cheson BD, Bennett JM, Kantarjian H, et al.: World Health Organization (WHO) international working group. Report of an international working group to standardize response criteria for myelodysplastic syndromes. Blood 2000, 96:3671–3674.

    PubMed  CAS  Google Scholar 

  32. Cheson BD, Greenberg PL, Bennett JM, et al.: Clinical application and proposal for modification of the International Working Group (IWG) response criteria in myelodysplasia. Blood 2006, 108:419–425.

    Article  PubMed  CAS  Google Scholar 

  33. National Comprehensive Cancer Network: NCCN Clinical Practice Guidelines in oncology: myelodysplastic syndromes. Available at http://www.nccn.org. Accessed April 2008.

  34. Aul C, Schneider W: The role of low-dose cytosine arabinoside and aggressive chemotherapy in advanced myelodysplastic syndromes. Cancer 1989, 64:1812–1818.

    Article  PubMed  CAS  Google Scholar 

  35. Oosterveld M, Wittebol SH, Lemmens WA, et al.: The impact of intensive antileukaemic treatment strategies on prognosis of myelodysplastic syndrome patients aged less than 61 years according to International Prognostic Scoring System risk groups. Br J Haematol 2003, 123:81–89.

    Article  PubMed  Google Scholar 

  36. Saunthararajah Y, Nakamura R, Nam JM, Robyn J: HLA-DR15 (DR2) is overrepresented in myelodysplastic syndrome and aplastic anemia and predicts a response to immunosuppression in myelodysplastic syndrome. Blood 2002, 100:1570–1574.

    PubMed  CAS  Google Scholar 

  37. Jonásova A, Neuwirtová R, Cermák J, et al.: Cyclosporin A therapy in hypoplastic MDS patients and certain refractory anaemias without hypoplastic bone marrow. Br J Haematol 1998, 100:304–309.

    Article  PubMed  Google Scholar 

  38. Appelbaum FR, Anderson J: Allogeneic bone marrow transplantation for myelodysplastic syndrome: outcomes analysis according to IPSS score. Leukemia 1998, 12(Suppl 1):S25–S29.

    PubMed  Google Scholar 

  39. Anderson JE, Appelbaum FR, Fisher LD, et al.: Allogeneic bone marrow transplantation for 93 patients with myelodysplastic syndrome. Blood 1993, 82:677–681.

    PubMed  CAS  Google Scholar 

  40. Runde V, de Witte T, Arnold R, et al.: Bone marrow transplantation from HLA-identical siblings as first-line treatment in patients with myelodysplastic syndromes: early transplantation is associated with improved outcome. Chronic Leukemia Working Party of the European Group for Blood and Marrow Transplantation. Bone Marrow Transplant 1998, 21:255–261.

    Article  PubMed  CAS  Google Scholar 

  41. Silverman LR, Demakos EP, Peterson BL, et al.: Randomized controlled trial of azacitidine in patients with the myelodysplastic syndrome: a study of the Cancer and Leukemia Group B. J Clin Oncol 2002, 20:2429–2440.

    Article  PubMed  CAS  Google Scholar 

  42. Kornblith AB, Herndon JE II, Silverman LR, et al.: Impact of azacytidine on the quality of life of patients with myelodysplastic syndrome treated in a randomized phase III trial: a Cancer and Leukemia Group B study. J Clin Oncol 2002, 20:2441–2452.

    Article  PubMed  CAS  Google Scholar 

  43. Kantarjian H, Issa JP, Rosenfeld CS, et al.: Decitabine improves patient outcomes in myelodysplastic syndromes: results of a phase III randomized study. Cancer 2006, 106:1794–1803.

    Article  PubMed  CAS  Google Scholar 

  44. Saba HI, Wijermans PW: Decitabine in myelodysplastic syndromes. Semin Hematol 2005, 42(3 Suppl 2):S23–S31.

    Article  PubMed  CAS  Google Scholar 

  45. Bartlett JB, Dredge K, Dalgleish AG: The evolution of thalidomide and its IMiD derivatives as anticancer agents. Nat Rev Cancer 2004, 4:314–322.

    Article  PubMed  CAS  Google Scholar 

  46. List A, Dewald G, Bennett J, et al.: Myelodysplastic Syndrome-003 Study Investigators: lenalidomide in the myelodysplastic syndrome with chromosome 5q deletion. N Engl J Med 2006, 355:1456–1465.

    Article  PubMed  CAS  Google Scholar 

  47. List AF, Dewald G, Bennett J, et al.: Clinical benefit from 2 phase II trials evaluating lenalidomide (REVLIMID) in lower-risk myelodysplastic syndrome patients with or without del 5q cytogenetic abnormalities [abstract 1032]. Presented at the 11th Congress of the European Hematology Association; June 15–18, 2006; Amsterdam, The Netherlands.

  48. List A, Kurtin S, Roe DJ, et al.: Efficacy of lenalidomide in myelodysplastic syndromes. N Engl J Med 2005, 352:549–557.

    Article  PubMed  CAS  Google Scholar 

  49. Kuendgen A, Strupp C, Aivado M, et al.: Treatment of myelodysplastic syndromes with valproic acid alone or in combination with all-trans retinoic acid. Blood 2004, 104:1266–1269.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Hematology/Oncology, University of California, Los Angeles, Box 957059, Suite 2333 PVUB, Los Angeles, CA, 90095, USA

    Ahrin Koppel

Authors
  1. Ahrin Koppel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Gary Schiller
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ahrin Koppel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Koppel, A., Schiller, G. Myelodysplastic syndrome: An update on diagnosis and therapy. Curr Oncol Rep 10, 372–378 (2008). https://doi.org/10.1007/s11912-008-0058-z

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11912-008-0058-z

Keywords

Search

Navigation