گزارش يك مورد ترومبوز مزمن وريد پورت 2 سال بعد از طحال برداري در بيمار بتاتالاسمي ماژور

A CASE REPORT OF 2 YEARS POST SPLENETOMY CHRONIC VEIN THROMBOSIS IN BETH THALASSEMIA MAJER

Objectives:Splenectomy sometimes needed in the path of longterm treatment of Thalassemia disease. Post Spleneetoi Portal vein thrombosis is a complication of splenectomy , one of the interesting cases with the aim faster diagnosis and management have been discussed here. Case Report : The patient is a 23 years old female with B-thalassemia major, who was admitted in Thalassemia ward , with complaints of fever , limb and, abdominal pain ,dysuria and diarrhea. She had history of splenectomy ( 2 years ago ), cholecystectomy (1.5 years ago) and diabetes mellitus. In the first and second gray scale ultrasonography which performed in the first and second weeks of admission except hepatomegaly due to iron deposition otherwise was unremarkable Portal and splenic venous thrombosis with cavernous formation with extension to superiormesentric vein was noted, in third grayscale ultra -sonography which performed 3 days after second ultrasonography.These findings were confirmed with colour Doppler ultrasonography. In MRA , vascular network around stomach(endoscopy showed gastric fundal varices) in addition to extensive cavernous formation around portal vein was seen. For primary prevention of bleeding due to gastric varices propranolol equivalent to 1mg/kg/day was started. Discussion and conclusion :In major B-thalasemia patients, portal vein thrombosis have been seen after splenectomy , as two distinct acute and chronic forms. Due to hypercoagulability state(thrombocytosis,leukocytosis and increased rigidity of RBC) and splenic vein ectasia ( due to splenomegaly) possibility of portal vein thrombosis in major B- thalassemic patients is high. Abdominal CT Scan with contrast is the best diagnostic method.In acute phase anticoagulative drugs(heparin, warfarin) , thrombolytic drugs(t-PA) are useful. But in chronic phase(which is characterize with cavernous formation) a primary or secondary prevention of bleeding due to varices is regarded .