Myelofibrosis of the facial bones

Idiopathic myelofibrosis is a hematologic myeloproliferative disorder characterized by progressive marrow fibrosis, extramedullary hematopoiesis, and a leukoerythroblastic anemia. Consequently there are frequent secondarily precipitated multiple systems problems. This is the first report of this entity presenting with orofacial findings. The histopathogenesis, clinical presentation, and the therapeutic goals of treatment are reviewed.