Myelodysplastic Syndrome Differential Diagnoses

  • Author: Emmanuel C Besa, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP   more...
 
Updated: Oct 10, 2011
 
 

Differential Diagnoses

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Contributor Information and Disclosures
Author

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Coauthor(s)

Ulrich Josef Woermann, MD  Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland

Disclosure: Nothing to disclose.

Chief Editor

Koyamangalath Krishnan, MD, FRCP, FACP  Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians

Disclosure: Nothing to disclose.

Additional Contributors

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

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  2. Goldberg H, Lusk E, Moore J, Nowell PC, Besa EC. Survey of exposure to genotoxic agents in primary myelodysplastic syndrome: correlation with chromosome patterns and data on patients without hematological disease. Cancer Res. Nov 1 1990;50(21):6876-81. [Medline]. [Full Text].

  3. Kristinsson SY, Bjorkholm M, Hultcrantz M, et al. Chronic immune stimulation might act as a trigger for the development of acute myeloid leukemia or myelodysplastic syndromes. J Clin Oncol. Jul 20 2011;29(21):2897-903. [Medline]. [Full Text].

  4. Ma X, Does M, Raza A, Mayne ST. Myelodysplastic syndromes: incidence and survival in the United States. Cancer. Apr 15 2007;109(8):1536-42. [Medline]. [Full Text].

  5. Rollison DE, Hayat M, Smith M, et al. First report of national estimates of the incidence of myelodysplastic syndromes and chronic myeloproliferative disorders from the U.S. SEER program [abstract 247]. Blood. 2006;108:77a. [Full Text].

  6. Rollison DE, Howlader N, Smith MT, 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. Jul 1 2008;112(1):45-52. [Medline]. [Full Text].

  7. Bennett JM, Catovsky D, Daniel MT, et al. Proposed revised criteria for the classification of acute myeloid leukemia. A report of the French-American-British Cooperative Group. Ann Intern Med. Oct 1985;103(4):620-5. [Medline].

  8. Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman 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. Dec 1999;17(12):3835-49. [Medline]. [Full Text].

  9. Vardiman JW, Thiele J, Arber DA, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. Jul 30 2009;114(5):937-51. [Medline]. [Full Text].

  10. General Information About Myelodysplastic Syndromes. National Cancer Institute. Available at http://www.cancer.gov/cancertopics/pdq/treatment/myelodysplastic/HealthProfessional/page1#Reference1.9.

  11. Molldrem JJ, Leifer E, Bahceci E, et al. Antithymocyte globulin for treatment of the bone marrow failure associated with myelodysplastic syndromes. Ann Intern Med. Aug 6 2002;137(3):156-63. [Medline]. [Full Text].

  12. Greenberg P, Cox C, LeBeau MM, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. Mar 15 1997;89(6):2079-88. [Medline]. [Full Text].

  13. 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. May 15 2002;20(10):2429-40. [Medline]. [Full Text].

  14. List A, Kurtin S, Roe DJ, et al. Efficacy of lenalidomide in myelodysplastic syndromes. N Engl J Med. Feb 10 2005;352(6):549-57. [Medline]. [Full Text].

  15. Musto P, Lanza F, Balleari E, et al. Darbepoetin alpha for the treatment of anaemia in low-intermediate risk myelodysplastic syndromes. Br J Haematol. Jan 2005;128(2):204-9. [Medline].

  16. Jädersten M, Montgomery SM, Dybedal I, Porwit-MacDonald A, Hellström-Lindberg E. Long-term outcome of treatment of anemia in MDS with erythropoietin and G-CSF. Blood. Aug 1 2005;106(3):803-11. [Medline]. [Full Text].

  17. [Best Evidence] 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. Mar 2009;10(3):223-32. [Medline].

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Blood film (1000× magnification) demonstrating a vacuolated blast in a refractory anemia with excess of blasts in transformation. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
This bone marrow film (400× magnification) demonstrates an almost complete replacement of normal hematopoiesis by blasts in a refractory anemia with an excess of blasts in transformation. Note the signs of abnormal maturation such as vacuolation, double nucleus, and macrocytosis. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
Bone marrow film (1000× magnification) demonstrating ring sideroblasts in Prussian blue staining in a refractory anemia with excess of blasts in transformation. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
Bone marrow film (1000× magnification) demonstrating granular and clotlike positive reaction in periodic acid-Schiff staining in a refractory anemia with excess of blasts in transformation. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
Table 1. International Prognostic Scoring System Risk Groups and Prognosis[10]
Risk GroupTime to Development of AML (y)Median Survival (y)
Low risk9.45.7
Intermediate risk – 13.33.5
Intermediate risk – 21.11.2
High risk0.20.4
AML – Acute myelogenous leukemia
Table 2. IPSS Score for Staging MDS[12]
Prognostic Variable0 Points0.5 Points1 Point1.5 Points2 Points
Bone marrow blasts, %< 55-10–11-2021-30
Karyotype*GoodIntermediatePoor––
Cytopenias0/12/3–––
*Good is no abnormality (46,XX or 46,XY), -Y, del(5q), del(20q); intermediate is other abnormalities, such as trisomy 8 (+8); and poor is complex (33 abnormalities or chromosome 7 abnormality [ie, 7q- or -7]).
Table 3. Categories of FAB Classification Versus WHO Classification for Myelodysplastic Syndrome (MDS)
FAB



Classification



WHO-2004



Classification



WHO-2008



Classification



RARA RCMD 5q-RCUD RCMD 5q-
RARSRARS RCMD-RSRARS RCMD-RS RARS-T
RAEBRAEB-1 RAEB-2RAEB-1 RAEB-2
CMMLCMML-1 CMML-2CMML-1 CMML-2
RAEB-TAMLAML
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