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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 John M. Bennett, MD University of Rochester Medical CenterHematomorphologistChair, MDS Foundation
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 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 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.
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 • 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 • 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