Myelofibrosis and Portal Hypertension: The Case for Primary Variceal Screening

Myelofibrosis is a hematologic condition that predisposes to the formation of large and small portal venous clots. Portal injury is believed to underlie the mechanism of development of noncirrhotic portal hypertension in this population. We describe a patient with myelofibrosis, proven portal hypertension, and extramedullary hematopoiesis with no imaging or pathologic evidence of microvascular or macrovascular portal clot. We provide a concise review of the literature which highlights that patients with myelofibrosis and related conditions of polycythemia vera and essential thrombocytosis present not infrequently with portal hypertension and variceal bleeding. We propose this population may benefit from primary variceal screening.