Characteristics and Outcome of Primary Budd Chiari Syndrome due to Myeloproliferative Disorder in Egyptian Patients, A Single Center Study

Abstract

Abstract Background Budd–Chiari syndrome (BCS) is a rare life-threatening disorder caused by obstruction of the hepatic venous outflow tract at any level between the small hepatic veins and the right atrium, hence also known as hepatic venous outflow tract obstruction (HVOTO). Primary BCS is often related to multiple thrombophilic conditions, including primary myeloproliferative disorders. Aim of the Work The present study aimed to assess characteristics of Primary Budd Chiari syndrome due to Myeloproliferative disorder in Egyptian patients and the outcome of the radiologic endovascular intervention (TIPS or angioplasty) Patients and Methods This was a retrospective cohort study that was carried out in The Tropical Medicine department, at Ain Shams University Hospitals. A total of 37 patients were enrolled, including 12 males and 25 females, with an age of 15-49 years (mean: 32 years) with primary BCS and MPDs during the interval between January 2006 and March 2023. Results JAK2 was positive in 64.9% of BCS patients, more in ET patients. ET was more frequent in BCS due to MPDs. Furthermore, these results validate routine inclusion of JAK2V617F in the diagnostic workup of BCS. Rate of stent occlusion was high 33.3% due MPDs thrombophilic burden nature. Conclusion MPDs are major aetiology of BCS. TIPS placement is an effective treatment for portal hypertension, but in any case, long-term high intensity anticoagulation treatment is essential in order to prevent recurrent thrombosis.