Diagnosis and Treatment of Myelofibrosis
Diagnosis and Treatment of Myelofibrosis
June 2016
- A 72-year old male presents to his primary care physician with fatigue, abdominal pain lasting 3 months, and night sweats
- PMH includes depression and gout
- Physical Exam: BP, 155/85; spleen palpable 6 cm below costal margin
- Laboratory values:
- Hb= 9.8 g/L
- HCT= 38%
- WBC= 22.3 x109/L
- Platelets= 255 x109/L
- Bone marrow biopsy:
- Megakaryocytic proliferation and atypia with evidence of reticulin fibrosis
- Blood smear showed leukoerythroblastosis
- Genetic testing showsJAK2 V617Fmutation
- Diagnosis: Primary myelofibrosis
NCCN guidelines indicate rusfertide for patients who are intolerant to phlebotomy or who receive frequent phlebotomy, regardless of risk status.
The FDA designation is for LYT-200 plus a hypomethylating agent for relapsed/refractory high-risk myelodysplastic syndromes.
The application for bezuclastinib in advanced systemic mastocytosisis is supported by data from the APEX trial.