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MDS Classification and Prognosis. John M. Bennett, MD University of Rochester Medical Center Hematomorphologist Chair, MDS Foundation. Primary Myelodysplastic Syndromes. Malignant disorders characterized by Ineffective hematopoiesis (≥ 1 lineage) Variable % of leukemic blasts

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mds classification and prognosis

MDS Classification and Prognosis

John M. Bennett, MD

University of Rochester Medical CenterHematomorphologistChair, MDS Foundation

primary myelodysplastic syndromes
Primary Myelodysplastic Syndromes
  • Malignant disorders characterized by
    • Ineffective hematopoiesis (≥ 1 lineage)
    • Variable % of leukemic blasts
  • Median age is 70
  • 30% progress to AML
  • US incidence: ~15,000 cases annually
  • US prevalence: 35,000 to 55,000
  • Majority present with moderate to severe anemia
  • Del 5q is associated with transfusion-dependent refractory anemia
international prognostic scoring system
International Prognostic Scoring System

All 3 prognostic variables required to generate IPSS score

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

ipss risk category correlates with mds survival outcomes
IPSS Risk Category Correlates With MDS Survival Outcomes

100

80

del(5)(q13q33)

60

Percent surviving

40

Low

20

Int-1

Int-2

High

0

0 2 4 6 8 10 12 14 16 18 20 22 24 26 28 30

Time, yr

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

management of low int 1 risk mds
Treatment:

Recombinant erythroid growth factors

5-azacytidine (Vidaza®)

Transfusions

Management of Low/Int-1-Risk MDS

Patients with MDS present with anemia and fatigue

transfusions an imperfect solution
Transfusions: An Imperfect Solution
  • Transient Hct improvement
  • Hct not restored to normal
  • Associated morbidities
    • Iron overload (250 mg iron/unit)
      • Unable to be phlebotimized
      • Requires chelation
    • Infectious diseases
    • Transfusion reactions
  • Demand on blood supply
  • Impact on patients’ lives
impact of lenalidomide on a patient
Impact of Lenalidomide on a Patient
  • Patient #014-3002
    • 84-yr-old female with Low-Risk MDS; del 5q
    • Required 116 RBC units over 54 mo
      • EPO resistant
      • Chelation therapy for iron overload
    • Started lenalidomide: Dec 03 (Hgb: 8.2 g %)
    • Last unit transfused: Feb 04; Hgb: 10.0 g % by Day 50
    • Hgb 13.3 g % by Apr 04
    • Has remained at that level to present
    • Tolerating phlebotomy for iron overload
    • Marrow morphology and cytogenetics normalized
    • Remains on study in complete remission