RESPONSE Trial and Hydroxyurea Resistance

Opinion
Video

Dr. Kuykendall comments on the high rates of hematocrit control and spleen response with ruxolitinib vs standard therapy in the RESPONSE trial for hydroxyurea-resistant/intolerant PV, and how these efficacy findings inform his treatment choices.

Case: Management of Advanced Polycythemia Vera in a 67-Year-Old-Male

Clinical Presentation:

  • 67-year-old male presented with frequent headache and dizziness
  • Prior medical history is notable for smoking.
  • PMH: unremarkable, no splenomegaly

Initial Clinical Workup and Diagnosis:

  • Labs:
    • Hemoglobin, 20.5 g/dL
    • White blood cell count, 13 K/L
    • Platelet count, 380 K/L
    • Hematocrit, 68%
  • JAK2-V617F mutation- variant allele frequency of 65%
  • Bone marrow biopsy: trilineage proliferation and pleiomorphic megakaryocytes
  • The patient started phlebotomy, aspirin, and Hydroxyurea (HU)

Initial Treatments:

  • The patient started phlebotomy, aspirin and HU.
    • Over the next year the patient underwent several phlebotomies and remained on aspirin and 2000 mg/day of HU

Disease Progression and Subsequent Treatments:

February 2019

  • 2 phlebotomies since the last visit 3 months earlier
  • He is also experiencing fatigue and night sweats.
  • Labs
    • Hgb, 15.5 g/dL
    • WBC, 13 K/L
    • Platelet count, 380 K/L
    • Hematocrit, 47%
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