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| Cirrhosis on Budd-Chiari syndrome revealing a Polycythemia Vera: A case report
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Ahlam EL Ghmari, Fatima Benahsin, Samia Bentabet, Asmae Lamine, Maria Lahlali, Hakima Abid, Amine El Mekkaoui, Mounia El Yousfi, Sidi Adil Ibrahimi, Dafr-Allah Benajah, Nada Lahmidani
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Abstract:
Introduction: Polycythemia Vera (PV) is the most common etiology of primary Budd-Chiari syndrome (BCS), with a prevalence of 30%-50%. This association poses a diagnostic problem, because the abnormalities of the hemogram during PV are masked by hypersplenism, and a therapeutic problem due to the management of anticoagulants in cirrhotic patients. The aim of this work is to demonstrate the diagnostic and therapeutic particularities of SBC at the stage of cirrhosis associated with PV through a medical observation collected in the hepato-gastroenterology department of University Hospital , FEZ. Observation: This is a 42 year old man, with no medical past history, admitted to our department for exploration of ascitis. The underlying etiology was a cirrhosis on BCS, confirmed by the visualization of thrombosis of the right and median hepatic veins on abdominal angio CT scan . The etiological investigation of the BCS revealing PV, suspected on the results of the haemogram and confirmed by an osteo-medullary biopsy and by the presence of the V617F mutation of the JAK 2 gene by allele-specific PCR. An anticoagulant treatment, based on low molecular weight heparin and anti-vitamins K, was started and the patient was treated with cytoreductive therapy with satisfactory clinico-biological evolution. Conclusion: Primary SBC is often due to PV, but in the majority of cases, the haemogram is not characteristic, mainly because of the associated hypersplenism, therefore the systematic search for the JAK2 mutation. Treatment is based essentially on anticoagulants, which require special management in cirrhotic patients because of the risk of bleeding due to portal hypertension.
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