RESPONSE Trial and Hydroxyurea Resistance

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%