Factors Considered for Initial Therapy in Myelofibrosis

Opinion
Video

Dr. Bose shares his perspective on the factors he considers when deciding on initial therapy for myelofibrosis, including his approach to timing of treatment initiation and how splenomegaly influences his choice of JAK inhibitors.

Case: Intermediate-Risk Primary Myelofibrosis in a 68-Year-Old Woman

Clinical Presentation:

  • A 68-year-old woman presented to her physician with symptoms of mild fatigue.
  • Spleen was palpable 6-7 cm below the left costal margin.

PM: No known comorbidities

Initial Clinical Workup and Diagnosis:

  • NGS Testing: JAK2 V617F mutation
  • Karyotype: 46XX
  • BM biopsy: megakaryocyte proliferation and atypia with evidence of reticulin fibrosis
  • Blood smear: leukoerythroblastosis
  • Diagnosis: Primary myelofibrosis
  • Risk:
    • DIPSS: Intermediate-2
    • Mipss70: Intermediate risk
  • Lab Values
    • RBC 3.40 x 1012/L
    • Hgb 9.7 g/dL
    • HCT 32.3%
    • MCV 94 fL
    • WBC 23.0 x 109/L
    • PLT 450 x 109/L
    • PB Blasts <1%

Initial Treatments:

  • Initiated on ruxolitinib.
    • The patient was not interested in transplant.
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