Hydroxyurea Resistant Polycythemia Vera

January 2015

• A 39-year-old male presents with headache and weight loss
• He is a 2-pack a day smoker
• PMH includes type 2 diabetes, moderately controlled on medication; newly-diagnosed hypertension
• Physical exam: BP, 176/94, otherwise unremarkable
• Laboratory values:
  • Hb; 233 g/L
  • Hct; 68.9%;
  • Mean corpusc. vol.; 81 fL
  • Leukocytes; 4.4 × 10⁹/L
  • Platelets; 145 × 10⁹/L
• Bone marrow biopsy;
  • MF-3 fibrosis and megakaryocytic hyperplasia with atypia
  • Normal karyotype
  • JAK2-positive
• Patient was started on phlebotomy as needed and aspirin

April 2015

• Patient had 3 phlebotomies in the last 3 months.
• He reported increasing dizziness, headaches and nausea
• He was continued on phlebotomy as needed; aspirin was continued
• Patient was started on Hydroxyurea 1000 mg daily

July 2015

• Patient comes back 3 months later, he has had 2 phlebotomies
• Hydroxyurea is increased to 1500 mg

October 2015

• Patient returns 3 months later with pruritus, still requiring phlebotomy
• He is started on hydroxyurea 2000 mg daily

January 2016

• The patient has had 2 phlebotomies since his last visit; he reports abdominal fullness
• He also has restless legs and is complaining that food tastes funny
• Spleen is palpable 7 cm below costal margin