Skip to Main Content
Home > Books > Hematology-Oncology Therapy, 2e >

Chapter 67: Myelodysplastic Syndromes

Michael M. Boyiadzis; Neal S. Young

  • View
  • Print
  • Get Citation

    Citation

    AMA Citation
    Boyiadzis MM, Young NS. Boyiadzis M.M., Young N.S. Boyiadzis, Michael M., and Neal S. Young.Myelodysplastic Syndromes. In: Boyiadzis MM, Frame JN, Kohler DR, Fojo T. Boyiadzis M.M., Frame J.N., Kohler D.R., Fojo T Eds. Michael M. Boyiadzis, et al.eds. Hematology-Oncology Therapy, 2e New York, NY: McGraw-Hill; . http://hemonc.mhmedical.com/content.aspx?bookid=1611&sectionid=126670219. Accessed September 21, 2018.
    MLA Citation
    Boyiadzis MM, Young NS. Boyiadzis M.M., Young N.S. Boyiadzis, Michael M., and Neal S. Young.. "Myelodysplastic Syndromes." Hematology-Oncology Therapy, 2e Boyiadzis MM, Frame JN, Kohler DR, Fojo T. Boyiadzis M.M., Frame J.N., Kohler D.R., Fojo T Eds. Michael M. Boyiadzis, et al. New York, NY: McGraw-Hill, , http://hemonc.mhmedical.com/content.aspx?bookid=1611&sectionid=126670219.

    Download citation file:

    RIS (Zotero)
    EndNote
    BibTex
    Medlars
    ProCite
    RefWorks
    Reference Manager
    Mendeley
    © Copyright
  • Tools
  • Search Book
  • Top
  • Return
  • Clip

++

INTRODUCTION

++

Myelodysplastic syndromes (MDSs) are clonal disorders characterized initially by ineffective hematopoiesis and subsequently by the development of acute leukemias. Peripheral blood cytopenias in combination with a hypercellular bone marrow exhibiting dysplastic changes are the hallmark of MDS

++

Epidemiology

Table Graphic Jump Location
Favorite Table | Download (.pdf) | Print
Epidemiology
Incidence: Increases with Age
Mean age: 68 years Male-to-female ratio: 1:1
Overall: 4.1 per 100,000
Ages 50–59 years: 0.3 per 100,000
Ages 60–69 years: 15 per 100,000
Ages 70–79 years: 49 per 100,000
Ages >80 years: 89 per 100,000

 

Dunbar CE, Saunthararajah Y. Myelodysplastic syndromes. In: Young NS, ed. Bone Marrow Failure Syndromes. Philadelphia, PA: WB Saunders; 2000:69–98

Greenberg P et al. Blood 1997;89:2079–2088.

Erratum in: Blood 1998;91:1100. Comment in: Blood 1997;90:2843–2846, Blood 2001;98:1985

++

Work-up

CBC with differential

Serum liver function tests, electrolytes, serum creatinine

Bone marrow biopsy and aspiration with iron stains, flow cytometry, cytogenetics

HLA typing for patients who are candidates for allogeneic stem cell transplantation

RBC folate, serum B12, serum iron/TIBC/ferritin, serum erythropoietin level (prior to RBC transfusion)

HIV testing if clinically indicated

Thyroid function tests to rule out hypothyroidism

HLA-DR15 typing to assist determination of response to immunosuppressive therapy

Evaluate patients with chronic myelomonocytic leukemia (CMML) for 5q31–33 translocations and/or PDGFRβ gene rearrangements

JAK2 mutation in patients with thrombocytosis

 

Table Graphic Jump Location
Favorite Table | Download (.pdf) | Print
Frequent Chromosomal Aberrations in MDS
Numerical Translocations Deletions
Cytogenetics (%) Cytogenetics (%) Cytogenetics (%)
+ 8 19 inv 3 7 del 5q 27
−7 15 t (1;7) 2 del 11q 7
+21 7 t (1;3) 1 del 12q 5
−5 7 t (3;3) 1 del 20q 5
    t (6;9) <1 del 7q 4
    t (5;12) <1 del 13q 2

 

−, Loss of chromosome; +, additional chromosome; inv, inversion; t, translocation; del, deletion

  • Clonal cytogenetic abnormalities: 30–79%

  • Deletions are more frequent than translocations

++

Classification Systems for MDS

Table Graphic Jump Location
Favorite Table | Download (.pdf) | Print
Classification Systems for MDS
French-American-British Classification of MDS
FAB Subtype % of Bone Marrow Blasts % of Peripheral Blasts
Refractory anemia (RA) <1 <5
Refractory anemia with ringed sideroblasts (RARS) <1 <5
Refractory anemia with excess blasts (RAEB) <5 5–20
Refractory anemia with excess blasts in transformation (REAB-t) ≥5 21–30
Chronic myelomonocytic leukemia (CMML) (>1000 monocytes/μL blood) <5 5–20

 

Bennett JM et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 1982;51:189–199

 
2008 World Health Organization Classifi cation of MDS
Subtype Blood Bone Marrow
Refractory cytopenia with unilineage dysplasia (RCUD)✫ Single or bicytopenia Dysplasia in ≥10% of 1 cell line, <5% blasts
Refractory anemia with ring sideroblasts (RARS) Anemia, no blasts ≥15% of erythroid precursors w/ring sideroblasts, erythroid dysplasia only, <5% blasts
Refractory cytopenia with multilineage dysplasia (RCMD) Cytopenias, monocytes <1000/μL Dysplasia in ≥10% of cells in ≥2 hematopoietic lineages, ±15% ring sideroblasts, <5% blasts
Refractory anemia with excess blasts-1 (RAEB-1) Cytopenias, ≤2–4% blasts, monocytes <1000/μL Unilineage or multilineage dysplasia, no Auer ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.

This site uses cookies to provide, maintain and improve your experience. MHE Privacy Center Close