Acute portal vein thrombosis in a 59-year-old male with JAK2 V617F mutation

Abstract

Portal vein thrombosis is an uncommon finding that typically arises in the context of cirrhosis. In the acute setting, it may present with abdominal pain, portal hypertension, ascites, gastrointestinal bleeding, or mesenteric ischemia. Local risk factors that predispose its formation include: cirrhosis, hepatocellular carcinoma, pancreatitis, and intraabdominal infection. Systemic factors, including hypercoagulable states and sepsis, also pose an increased risk. JAK2 V617F positive myeloproliferative disorders are associated with systemic prothrombotic states and are a less frequently identified cause of portal vein thrombosis. We present a case of acute unprovoked portal vein thrombosis diagnosed in a 59-year-old male without local disease factors. Computed tomography, magnetic resonance cholangiopancreatography, and ultrasound demonstrated the presence of portal vein thrombosis with neighboring periportal and pancreatic head edema. Peripheral blood testing detected the presence of JAK2 V617F mutation. The patient was discharged on 6-month anticoagulation therapy and outpatient follow-up.

Keywords: JAK2 V617F; Portal vein thrombosis.

Fig. 1

IV contrast enhanced CT images with axial (A, B) and coronal (C, D) depict decreased attenuation in the portal vein consistent with thrombosis (white arrows) with axial and coronal views. Reticulation of the neighboring periportal fat to include the peripancreatic fat indicates edema. Incidentally noted is a large left renal cyst.

Fig. 2

T2-weighted spectral attenuated inversion recovery (SPAIR) MRI axial images (E-H) reveal increased signal in the portal vein indicative of thrombosis (dashed arrows) and increased signal surrounding the vein consistent with edema. Incidentally noted is a large left renal cyst.

Fig. 3

Portal vein ultrasound images (I, J) without and with color Doppler demonstrate increased echogenicity and no flow within the portal vein consistent with portal vein thrombosis.

Fig. 4

Six months after the initial imaging, IV contrast enhanced CT images in axial (K-N) and coronal (O, P) plane depict decreased attenuation in the portal vein consistent with thrombosis (white arrows). Tubular enhancing structures around the thrombosed portal vein are consistent with cavernous transformation of the portal vein (yellow arrows). Collateral vessels are noted to surround the gall bladder demonstrated in image N. (Color version of this figure is available online.)