Myelodysplastic syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]Amandeep Singh M.D.[3]

Overview

If left untreated, a high percentage of patients with myelodysplastic syndrome may progress to develop acute myeloid leukemia or die due to bone marrow failure. Common complications of myelodysplasia include progression to acute myeloid leukemia, bone marrow failure, infection, hemorrhage, and iron overload. Prognosis is generally poor, and the 5-year survival rate of patients with high IPSS score myelodysplastic syndrome is approximately 55%.

Natural history

If left untreated, patients with myelodysplastic syndrome may progress to develop weight loss, bone marrow failure, infection, hemorrhage, and iron overload.[1]

Complications

Common complications of myelodysplasia include:[1]

Prognosis

Prognosis is generally poor, and the 5-year survival rate of patients with high IPSS score myelodysplastic syndrome is approximately 55%. A variety of pathologic and risk classification systems have been developed to predict the overall survival of patients with myelodysplastic syndrome and the evolution from myelodysplastic syndrome to acute myeloid leukemia. Major prognostic classification systems include the International Prognostic Scoring System (IPSS), revised as the IPSS-R; the WHO Prognostic Scoring System (WPSS), and the MD Anderson Cancer Center Prognostic Scoring Systems. Clinical variables in these systems have included bone marrow and blood myeloblast percentage, specific cytopenias, transfusion requirements, age, performance status, and bone marrow cytogenetic abnormalities.[6]

IPSS

The IPSS incorporates bone marrow blast percentage, number of peripheral blood cytopenias, and cytogenetic risk group.[6]

IPSS-R

Compared with the IPSS, the IPSS-R updates and gives greater weight to cytogenetic abnormalities and severity of cytopenias, while reassigning the weighting for blast percentages.[6]

WPSS

In contrast to the IPSS and IPSS-R, which should be applied only at the time of diagnosis, the WPSS is dynamic, meaning that patients can be reassigned categories as their disease progresses.[6]

References

  1. 1.0 1.1 Natelson, Ethan A.; Pyatt, David (2013). "Acquired Myelodysplasia or Myelodysplastic Syndrome: Clearing the Fog". Advances in Hematology. 2013: 1–11. doi:10.1155/2013/309637. ISSN 1687-9104.
  2. Weisdorf DJ, Oken MM, Johnson GJ, Rydell RE (December 1983). "Chronic myelodysplastic syndrome: short survival with or without evolution to acute leukaemia". Br. J. Haematol. 55 (4): 691–700. PMID 6608368.
  3. Pomeroy C, Oken MM, Rydell RE, Filice GA (March 1991). "Infection in the myelodysplastic syndromes". Am. J. Med. 90 (3): 338–44. PMID 2003516.
  4. Bennett JM (November 2008). "Consensus statement on iron overload in myelodysplastic syndromes". Am. J. Hematol. 83 (11): 858–61. doi:10.1002/ajh.21269. PMID 18767130.
  5. Gattermann N (July 2008). "Overview of guidelines on iron chelation therapy in patients with myelodysplastic syndromes and transfusional iron overload". Int. J. Hematol. 88 (1): 24–29. doi:10.1007/s12185-008-0118-z. PMC 2516534. PMID 18581200.
  6. 6.0 6.1 6.2 6.3 Prognostic Scoring Systems of myelodysplastic syndrome. National Cancer Institute (2015). http://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq/#link/_204_toc. Accessed on December 11, 2015

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