This is Part 3 of Updates in Myeloproliferative Neoplasms, a three-part video roundtable series.

 

In this video, Dr. Naveen Pemmaraju and Dr. Gabriela Hobbs discuss goals of care in primary myelofibrosis.

 

The patient is a 27-year-old woman who presents to the clinic after 6 months of unintentional weight loss, night sweats, and fatigue. On physical exam, she has splenomegaly that is palpable at 7 cm below the left costal margin. Lab tests show anemia, leukocytosis, 1% circulating blasts in her peripheral blood, and high LDH. Bone marrow biopsy reveals marked reticulum fibrosis corresponding with MF-3 on the WHO histological grading, JAK V617F mutation, and diploid cytogenetics, and she is diagnosed with primary myelofibrosis.

 

In the conversation that ensues, the faculty discuss initial treatment options for primary myelofibrosis, the role of JAK inhibitors, and how treatment goals are beginning to move beyond spleen and symptoms and to instead focus on disease modification. They review the current approved JAK inhibitors, as well as novel therapies and combinations that are on the horizon.