Future Management and Unmet Needs in Myelofibrosis

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.