PORTAL VEIN THROMBOSIS WITH PORTAL HYPERTENSION IN NEWLY DIAGNOSED MYELOPROLIFERATIVE NEOPLASM; A JUDICIOUS REFERRAL IN EMERGENCY DEPARTMENT

Abstract

Introduction: According to Centers for Disease Control and Prevention (CDC), abdominal pain is one of the principal reasons for presentation to the Emergency Department (ED). However, portal vein thrombosis (PVT) is an unusual cause of abdominal pain. On the other hand, a patient who frequently visited ED may have something to befall if we do not apprehend the case immediately.

Case: We report a case of PVT with portal hypertension secondary to a newly diagnosed myeloproliferative neoplasm (MPN) likely essential thrombocytosis in a previously healthy 27-year-old gentleman. He had multiple visits to our ED and was treated for acute dyspepsia with an outpatient scope appointment. During his third presentation, he complained of severe abdominal pain especially over the epigastric region and was admitted for prompt investigations and to rule out hematological malignancy.

Result: He had hepatosplenomegaly, haemoglobin of 15.1g/dL with hematocrit of 45.6%, leukocytosis 11.09 x109/L, thrombocytosis 864x109/L and high LDH 308 U/L. His liver function was normal. Inpatient ultrasound and CT abdomen showed chronic portal vein thrombosis with collaterals and cavernous transformations, splenic vein thrombosis and hepatosplenomegaly. Followed OGDS had revealed multiple fundal large varices. His JAX2 V617F mutation was positive. He was started on anticoagulant and beta-blocker to control the symptoms. Hydroxyurea was postponed due to worsening liver function during follow-up.

Discussion: MPN can be classified into polycythemia vera, essential thrombocytosis and myelofibrosis. Non-cirrhotic PVT is rare and the differential diagnosis is vast. In addition, PVT in MPD carries significant morbidity and mortality with the risk of multi organs involvements. 

Conclusion: Patients with multiple ED visits should be thoroughly investigated. Considering this case, it points out that a good history taking with the involvement of correct in-depth physical examinations and supportive workups are salient for better patient’s care. Early recognition, referral and prompt treatment are of paramount importance to stave off further complications.