JAK2 (V617F) Positive Latent Myeloproliferative Neoplasm Presenting with Splanchnic Vein Thrombosis

Myeloproliferative neoplasms (MPNs) are chronic clonal hematopoietic stem cell disorders characterized by proliferation of one or more of the granulocytic, red cell or platelet lineages in the bone marrow, with fairly normal maturation, resulting in increase in the leukocyte, erythrocytes and platelets in the blood. They also represent a common cause of splanchnic vein thrombosis (SVT). Herein, we describe a case of SVT as a presenting symptom of latent MPN. The patient has had normal complete blood counts since presentation. 3 ½ years later, she was found to have JAK2 (V617F) mutation and bone marrow biopsy was consistent with MPN. Five years later, her platelet count started to rise. In patients with a first episode of SVT, thrombophilia workup including JAK2 (V617F) mutation is warranted. Anticoagulation with heparin and warfarin is the treatment of choice for SVT.