myelodysplastic syndrome (MDS)

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Introduction

variable group of disorders with clonal proliferation of multipotential hematopoietic stem cells, with dysplasia & ineffective production of one or more cell lines.

Classification

* median survival in months

Etiology

Epidemiology

  • 80% of cases > 65 years of age[8]
  • men > women
  • 3.8 cases per 100,000 per year (age-adjusted)[10]

Pathology

Genetics

Clinical manifestations

Laboratory

* antiretroviral drugs can result induce macrocytosis

Diagnostic procedures

Complications

* major causes of death[1]

Differential diagnosis

Management

Prognosis

More general terms

More specific terms

Additional terms

References

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
    Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  2. Schiller G, in: UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
  3. WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. IARC Press 2001
  4. Estey & Schrier. Treatment and prognosis of the myelodysplastic syndromes. UpToDate 2004
  5. 5.0 5.1 Greenberg P et al International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997 Mar 15;89(6):2079-88. Erratum in: Blood 1998 Feb 1;91(3):1100. <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/9058730 <Internet> http://www.bloodjournal.org/cgi/content/full/89/6/2079 http://www.bloodjournal.org/cgi/content/abstract/89/6/2079 erratum Blood 91:1100, 1998. http://www.bloodjournal.org/cgi/content/full/91/3/1100
  6. deprecated reference
  7. 7.0 7.1 7.2 7.3 7.4 Steensma DP & Bennett JM The myelodysplastic syndromes: diagnosis and treatment Mayo Clin Proc 2006; 81:104 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16438486
  8. 8.0 8.1 8.2 Ma X, Does M, Raza A, Mayne ST. Myelodysplastic syndromes: incidence and survival in the United States. Cancer. 2007 Apr 15;109(8):1536-42. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17345612
    Ma X Epidemiology of myelodysplastic syndromes. Am J Med. 2012 Jul;125(7 Suppl):S2-5. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22735748
  9. 9.0 9.1 Raza A et al, Phase 2 study of lenalidomide in transfusion-dependent low- risk, and intermediate-1-risk myelodysplastic syndromes with karyotypes other than deletion 5q. Blood 2008, 111:86 PMID: https://www.ncbi.nlm.nih.gov/pubmed/17893227
  10. 10.0 10.1 Rollison DE et al. Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001-2004, using data from the NAACCR and SEER programs. Blood 2008 Jul 1; 112:45. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18443215
  11. 11.0 11.1 Tehranchi R et al. Persistent malignant stem cells in del(5q) myelodysplasia in remission. N Engl J Med 2010 Sep 9; 363:1025 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/20825315 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMoa0912228
  12. 12.0 12.1 ARUP Consult: Myelodysplastic Syndromes The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/myelodysplastic-syndromes
    ARUP consult: Myeloid Malignancies Mutation Panel by Next Generation Sequencing https://arupconsult.com/ati/myeloid-malignancies-mutation-panel-next-generation-sequencing
  13. 13.0 13.1 Foran JM, Shammo JM. Clinical presentation, diagnosis, and prognosis of myelodysplastic syndromes. Am J Med 2012; 125 (7 Suppl):S6-S13 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22735753
    Wang R, Gross CP, Halene S, Ma X. Comorbidities and survival in a large cohort of patients with newly diagnosed myelodysplastic syndromes. Leuk Res 2009; 33:1594-1598 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19324411
  14. 14.0 14.1 Fenaux P, Mufti GJ, Hellstrom-Lindberg E et al Efficacy of azacitidine compared with that of conventional care regimens in the treatment of higher-risk myelodysplastic syndromes: a randomised, open-label, phase III study. Lancet Oncol. 2009 Mar;10(3):223-32 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19230772
  15. 15.0 15.1 Borthakur G, Estey AE. Therapy-related acute myelogenous leukemia and myelodysplastic syndrome. Curr Oncol Rep. 2007 Sep;9(5):373-7. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17706165
  16. 16.0 16.1 List A, Dewald G, Bennett J, Giagounidis A et al Lenalidomide in the myelodysplastic syndrome with chromosome 5q deletion. N Engl J Med. 2006 Oct 5;355(14):1456-65. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17021321
  17. 17.0 17.1 Farah C, Bulai Livideanu C, Jegu J et al Prevalence and prognostic value of cutaneous manifestations in patients with myelodysplastic syndrome. J Eur Acad Dermatol Venereol. 2010 Oct;24(10):1171-5. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20202054
  18. Estey E. Acute myeloid leukemia and myelodysplastic syndromes in older patients. J Clin Oncol. 2007 May 10;25(14):1908-15. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17488990
  19. Greenberg PL, Tuechler H, Schanz J et al Revised international prognostic scoring system for myelodysplastic syndromes. Blood. 2012 Sep 20;120(12):2454-65. Epub 2012 Jun 27. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22740453
  20. Lyons RM Myelodysplastic syndromes: therapy and outlook. Am J Med. 2012 Jul;125(7 Suppl):S18-23 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22735747
  21. Vaughn JE, Scott BL, Deeg HJ. Transplantation for myelodysplastic syndromes 2013. Curr Opin Hematol. 2013 Nov;20(6):494-500 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24104409
  22. Michiels JJ, Berneman Z, Schroyens W, De Raeve H. Changing concepts of diagnostic criteria of myeloproliferative disorders and the molecular etiology and classification of myeloproliferative neoplasms: from Dameshek 1950 to Vainchenker 2005 and beyond. Acta Haematol. 2015;133(1):36-51. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25116092
  23. 23.0 23.1 Mufti GJ, Potter V. Myelodysplastic syndromes: who and when in the course of disease to transplant. Hematology Am Soc Hematol Educ Program. 2012;2012:49-55. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23233560
  24. Giagounidis A, Mufti GJ, Mittelman M et al Outcomes in RBC transfusion-dependent patients with Low-/ Intermediate-1-risk myelodysplastic syndromes with isolated deletion 5q treated with lenalidomide: a subset analysis from the MDS-004 study. Eur J Haematol. 2014 Nov;93(5):429-38. Epub 2014 Jun 9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24813620 Free PMC Article
  25. Nolte F, Angelucci E, Breccia M et al Updated recommendations on the management of gastrointestinal disturbances during iron chelation therapy with Deferasirox in transfusion dependent patients with myelodysplastic syndrome - Emphasis on optimized dosing schedules and new formulations. Leuk Res. 2015 Oct;39(10):1028-33. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26293555
  26. Fenaux P, Haase D, Sanz GF et al Myelodysplastic syndromes: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014 Sep;25 Suppl 3:iii57-69. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25185242
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  31. Myelodysplastic Syndromes (PDQ): Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/myelodysplastic/HealthProfessional

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