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Article

French

ID: <

10670/1.liarlh

>

Where these data come from
Budd-Chiari syndrome at cirrhosis stage revealing a Vaquez polyglobulie

Abstract

Introduction: The Polycythemia vera (PV) is the most common etiology of primary Budd-chiari syndrome (BCS), with a prevalence of 50%. This combination raises a diagnostic problem, as the blood cell counts anomalies in polycythemia vera are masked by hypersplenism (PV called occult), and therapeutic problem, due to the management of anticoagulation in patients with cirrhosis. The aim of our work is to highlight the diagnostic and therapeutic features of BCS in cirrhosis associated with PV, through a medical observation collated in the hepato-gastroenterology unit of the Ibn Rochd CHU in Casablanca. Observation: We report the case of a 67 year-old women patient, without specific medical history, admitted for balance of transudative ascites. The etiological balance of ascites is in favor of cirrhosis (Child Pugh B9) secondary to BCS, based on visualization with hepatic Doppler ultrasound of the middle and right hepatic vein thrombosis. The etiological balance of BCS reveals a polycythemia `np pagenum="186"/bvera, suspected in the data of the blood count and confirmed by a bone marrow biopsy and the presence of the V617F mutation of JAK 2 gene by PCR allele-specific. An anticoagulant therapy, based on low molecular weight heparin relayed by anti vitamin K, is started but the patient was lost sight. Conclusion: The primitive BCS is often secondary to PV but in the majority of cases, the blood cell count is not characteristic, especially if it is associated with hypersplenism, hence the systematic search of the JAK2. The treatment is mainly based on anticoagulants that require special management in cirrhotic patients because of bleeding risk associated with portal hypertension. The global prognosis is good (10 years survival: 80 %) providing a specialized and multidisciplinary management, involving the hematology, gastroenterology and cardiology is available. Nevertheless the risk of acute leukemia of PV and of hepatocellular carcinoma in cirrhotic patients must be kept in mind.

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