Primary deep vein thrombosis in the upper limb: A retrospective study with emphasis on pathogenesis and late sequelae

Thrombosis of the upper extremity (UEDVT) is an uncommon disease with an incidence of 2-3% of all deep vein thromboses. The aim of this study was to determine the frequency of thrombophilia, post-thrombotic symptoms (PTS), and the rate of complications and recurrences in patients with primary UEDVT, which includes idiopathic and effort-related thrombosis.

Thirty-two patients with primary UEDVT were participants in the study. All patients with malignancies and intravenous devices were excluded. Two different scoring instruments - the Villalta and the DASH - were used to diagnose PTS, and a visual analogue scale (VAS) was used to estimate pain and disability. To evaluate working capacity, an arm exercise test was performed. Blood samples were taken for antithrombin, protein C and S deficiencies, antiphospholipid antibodies, mutation of factor V, fibrinogen, D-dimer, and von Willebrand factor antigen.

None of the patients developed malignancy, pulmonary embolism, or recurrent UEDVT. Twenty-eight percent of the patients had mild to moderate PTS according to the scoring instruments. The arm exercise test and the VAS did not provide any additional information about the severity of PTS. The prevalence of thrombophilia was 40%; the most frequent disorders were the mutation of factor V (19%) and elevated fibrinogen (22%).

This study supports the belief that primary UEDVT is a benign disorder with a low risk for recurrence but with a high frequency of PTS. For a majority of the patients, the underlying cause of the thrombotic event is unclear.