Diagnostic and Therapeutic Challenges of Chronic Portal Vein Thrombosis Secondary to Myeloproliferative Neoplasm in Sub-Saharan Region: A Series of Cases and Literature Review

Abstract

Myeloproliferative neoplasms (MPN) are the commonest causes of non-cirrhotic abdominal vein thrombosis. MPN was observed in 30−40% with Budd Chiari Syndrome (BCS) and portal vein thrombosis (PVT), whereas this is the cause in only a minority of other types of venous thrombosis. Herein, we report 10 cases of chronic PVT secondary to Janus Kinase 2 gene (JAk 2) positive myeloproliferative neoplasm (MPN). The median age was 35 years with M:F 1:1. Portal hypertension was present at presentation in all of them. Nine patients have esophageal varices, of which 6 have bleeding at presentation. Classical MPN phenotype on complete blood count (CBC) was not present in 6 of them. Management of patients with anticoagulation was difficult with 4 patients experiencing bleeding during anticoagulation, and bleeding occurring despite the target international normalized ratio (INR) in 2 of them. Portal hypertension leading to hypersplenism and hemodilution may mask erythrocytosis and thrombocytosis, which are the main criteria used to diagnose MPN. The resulting absence of a typical MPN phenotype in routine laboratory studies alongside accessibility issues, and the need for a multidisciplinary treatment approach imparts a diagnostic as well as a therapeutic challenge for chronic PVT associated with Jak 2 positive MPN in sub-Saharan patients.