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A 60-year old man presented to our unit for iterative episodes of lipothymia and prominent superficial collateral venous circulation of the trunk (Figure A) after he was lost to follow-up for 4 years. He received 2 successive bone marrow transplantations in 2012 for dendritic cell leukemia. Heterozygous prothrombin G20210A mutation was found after a superior vena cava thrombosis from the implantable port occurred in 2015 with a recurrence in 2016 after anticoagulation was stopped. In 2017, a 12-cm long stent was implanted in the superior vena cava, and tinzaparin was given. The newly performed computed tomography scan evidenced a complete superior vena cava stent thrombosis (Figure B, yellow arrow) with important derivations joining the central venous circulation through femoral veins (Figure C, blue arrows) while he was still receiving tinzaparin. No other underlying cause was found. Rivaroxaban was started and the patient was discharged.
Superior vena cava syndrome is now rare and due to compression or thrombosis in 70% of cases linked to mediastinal malignancies (small cell carcinoma of the lung, lymphoma or metastasis). However, the remaining benign causes should be kept in mind (pacemaker wires, stents or catheters).
Indeed, these patients have a low risk of thrombophilia, and data are scarce to formally prescribe DOAC as no randomized control trial has been specifically conducted in patients with low-risk thrombophilia, even though a recent work found that, compared with vitamin K antagonists, DAOCs had equivalent efficacy and safety.
DOACs can be proposed after a recurrent thrombotic episode despite adequate anticoagulation with vitamin K antagonist or heparin, like our patient. Additional acquired thrombotic risk factors should trigger the discussion of a prolonged anticoagulation in patients with congenital thrombotic risk factors, such as any implantable material.
The superior vena cava syndrome: clinical characteristics and evolving etiology.