Approach to 1L Therapy Selection in LR-MDS

Opinion
Video

Jamie L. Koprivnikar, MD, presents a case of a 70-year-old man with SF3B1+ MDS with ring sideroblasts and discusses the approach to initial therapy in patients with lower-risk MDS who may have moderate-to-severe anemia.

Case: SF3B1+ MDS-RS: IPSS-M-Guided 1L Luspatercept

Clinical Presentation:

70-year-old man diagnosed 6 months ago with LR-MDS with multilineage dysplasia - moderate anemia (Hb 11.2) and thrombocytosis (PLT 500,000 μl)

  • SF3B1 mutation positive
  • Non-del(5q)
  • No family history of cancer or significant genotoxic agent exposure
  • IPSS-R: Low
  • IPSS-M: SF3B1α mutation [SF3B1, ASXL1 and TET2 mutations]

Current Visit Clinical Workup and Diagnosis:

  • Serum EPO - 250m U/L, Ring sideroblast (RS) agnostic (either positive or negative)
  • Hgb: 8.2 g/dL.
  • WBC and ANC: WNL
  • PLT: 450,000 μl

He complains of increasing fatigue over the past 1-2 months. He normally plays 2-3 rounds of golf a week. Lately he has only been playing a full round once a week and maybe 9 holes on another day.

Initial Treatment(s):

  • The patient was started on luspatercept at the starting dose [1.0 mg/kg] as 1L therapy.
  • After 24 weeks, the patient has not received any transfusion and his Hgb is now 10.7 g/dL.
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