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JAK Inhibitors in Myelofibrosis as Second Line Therapy and Beyond

CE / CME

Modern JAKi Strategies in Individualized Myelofibrosis Management: Current Practices for Second-line Therapy and Beyond

Physician Assistants/Physician Associates: 0.50 AAPA Category 1 CME credit

Nurse Practitioners/Nurses: 0.50 Nursing contact hour

Pharmacists: 0.50 contact hour (0.05 CEUs)

Physicians: maximum of 0.50 AMA PRA Category 1 Credit™

Released: March 14, 2025

Expiration: September 13, 2025

Pankit Vachhani
Pankit Vachhani, MD
CME/CE Information

Pretest

Progress
1 2 3
Course Completed
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1.

Case: Patient With MF (Second Line)



  • 68-yr-old woman diagnosed with PV in 2012; received therapy with ASA, hydroxyurea, and phlebotomy; history of irritable bowel syndrome

  • January 2023: Progressed to post-PV MF after presenting with erythrocytosis, splenomegaly, and B-symptoms

    • Began ruxolitinib 10 mg BID; had anemia requiring admissions and dizziness

    • TSS = 14



  • Assessment

    • Ultrasound: Maximum spleen length 22 cm (11 cm below costal margin)

    • PBC: WBC count: 35.5 x 109/L; hemoglobin: 7.1 g/dL; platelets: 70 x 109/L; 2% blasts

    • Biopsy: Consistent with PMF; 2+ reticulin fibrosis

    • Diagnostics: JAK2 mutation



Which of the following would you choose as the best therapeutic strategy for this patient?

2.

Case: Patient With AE on JAKi Therapy



  • 72-yr-old woman initially presents with shortness of breath, fatigue, significant night sweating, and weight loss

  • Assessment

    • Physical Exam: Splenomegaly (12 cm below costal margin)

    • PBC: WBC count 22 x 109/L, hemoglobin 9.9 g/dL, platelets 32 x 109/L, 1% blasts

    • Biopsy: Consistent with PMF; 2+ reticulin fibrosis

    • Diagnostics: JAK2 V617F, ASXL1, and TET2 mutations



  • She began pacritinib at 200 mg BID

  • During the first month of therapy, she reports grade 2 diarrhea which is impacting her quality of life but not causing dehydration

What is the most appropriate management strategy to address this adverse event?

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