Budd Chiari syndrome (BCS) and Myeloproliferative Neoplasms (MPN): A Moroccan Experience Center

Background and Objectives: Budd–Chiari syndrome is a vascular disorder of the liver which can cause fulminant liver injury and lethal portal hypertension-related complications. It is a rare disease and can be primary or secondary. The objective of our work is to detail evolution, treatment of patients with BCS and MPN according to the experience of a Moroccan center. Patients and Methods: This is a retrospective and descriptive study in the university hepato-gastroenterology department including all patients with BCS and MPN with portal hypertension (PH) over a period of 29 years. All our patients benefited from an etiological work-up and morphological explorations. Results: Out of a total 29 patients had BCS, 4 had MPN with a prevalence of 10%. Clinically, the signs of decompensated PH were predominant. Imaging confirmed BCS. The etiological work-up showed that all our patients had essential thrombocytemia. We had also association of other prothrombotic factors in 50 % of cases and a portal thrombosis in 25% of cases. Our patients had received treatment for the causative disease and treatment of thrombosis associated with the treatment of PH complications. The evolution was marked by the death of 2 patients (50%). Conclusion: The strong association between MPN and BCS is well established. The knowledge of the molecular mutations underlying MPN has dramatically improved in the last decade, allowing early diagnosis of MPN in a significant portion of BCS patient.