Aetiological factors of Budd-Chiari syndrome in Algeria

AIM: To study the clinical presentation of Budd-Chiari syndrome(BCS) and identify the aetiologies of this disease in Algeria. METHODS: Patients with BCS, hospitalised in our unit from January 2004 until June 2010 were included and the aetiological factors were assessed. Patients presenting a BCS in the setting of advanced-stage cirrhosis or a liver transplantation were excluded from the study. The diagnosis was established when an obstruction of hepatic venous outflow(thrombosis, stenosis or compression) was demonstrated. We diagnosed myeloproliferative disease(MPD) by bone marrow biopsy and V617 F JAK2 mutation. Anti-phospholipid syndrome(APLS) was detected by the presence of anticardiolipin antibodies, anti-β2 glycoprotein antibodies and Lupus anticoagulant. We also detected paroxysmal nocturnal haemoglobinuria(PNH) by flow cytometry. Celiac disease and Behcet disease were systematically investigated in our patients. Hereditary anticoagulant protein deficiencies were also assessed. We tested our patients for the G20210 A mutation at Beaujon Hospital. Imaging procedures were performed to determine a local cause of BCS, such as a hydatid cyst or a liver tumour.RESULTS: One hundred and fifteen patients were included. Mean follow up: 32.12 mo. Mean age: 34.41 years, M/F = 0.64. Chronic presentation was frequent:63.5%. The revealing symptoms for the BCS were ascites(74.8%) and abdominal pain(42.6%). The most common site of thrombosis was the hepatic veins(72.2%). Involvement of the inferior vena cava alone was observed in 3 patients. According to the radiological investigations, BCS was primary in 94.7% of the cases(n = 109) and secondary in 5.2%(n = 6). An aetiology was identified in 77.4% of the patients(n = 89); it was multifactorial in 27%(n = 31). The predominant aetiology of BCS in our patients was a myeloproliferative disease, observed in 34.6% of cases. APLS was found in 21.7% and celiac disease in 11.4%. Other acquired conditions were: PNH(n = 4), systemic disease(n = 6) and inflammatory bowel disease(n = 5). Anticoagulant protein deficiency was diagnosed in 28% of the patients(n = 18), dominated by protein C deficiency(n = 13). Secondary BCS was caused by a compressing hydatic cyst(n = 5) and hepatocellular carcinoma(n = 1). CONCLUSION: The main aetiologic factor of BCS in Algeria is MPD. The frequency of celiac disease justifies its consideration when BCS is diagnosed in our region.

Platelet donor database:a study on the specialist donor database for the patients with alloimmune thrombocytopenia in the Chinese population and the assessment of completeness

To the Editor:Due to the immunogenicity of the platelet antigens,especially blood group antigen(such as ABO antigens),human leukocyte antigen(HLA),human platelet antigen(HPA),and CD36(platelet glycoproteins IV),which can produce corresponding alloantibodies through immune factors such as blood transfusion,pregnancy,and drugs,the immune reaction of the platelet antigens and antibodies in patients will lead to various types of alloimmune thrombocytopenia.