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.

Keywords:

• portal cavernoma
• cholangiopathy
• Jak 2 mutation
• MPN
• Ethiopia
• sub-Sahara

Ethical Statement

The study was approved by the research ethics committee of Adera Medical Center. Consent to publish the cases and accompanying images has been obtained from all patients. The research is conducted in accordance with the Declaration of Helsinki.

Acknowledgments

We would like to extend our deepest thanks to the patients, their families, and our dear colleagues who participated in the care of the patients.

Disclosure

The authors report no conflicts of interest in this work.