SF3B1+ MDS-RS: IPSS-M-Guided 1L Luspatercept : Episode 1
Approach to 1L Therapy Selection in LR-MDS
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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