Catheter-related thrombosis associated with elevated factor VIII levels in cystic fibrosis

Abstract

We report a cystic fibrosis (CF) subject with extensive, central venous catheter-associated thrombosis and sustained elevation of factor VIII to levels normally associated with significantly increased risks of deep venous thrombosis. To determine the potential significance of this finding, the prevalence of elevated factor VIII levels in 22 adults with CF was investigated. Mean (S.D.) factor VIII level was 177 (43) U/dl, with 77% of subjects having levels >150 U/dl. The high prevalence of elevated factor VIII levels questions the significance of this finding in CF subjects with catheter-related thrombosis.

Central venous thrombosis and thrombophilia in cystic fibrosis: A prospective study

“…Inherited thrombophilia factors for venous thrombosis in adults [26] and children [27] fall mainly into two categories: hereditary antithrombin, protein C or S deficiencies, and factor V Leiden or prothrombin G20210A mutations. We found an unusually high prevalence of these abnormalities compared to healthy populations [26,27], but this was in agreement with previous publications on thrombophilia in CF [14][15][16][17]. In our study, the laboratory thrombophilic panel did not assess factors identified as hypothetic risk factors for thrombosis in the general population and possibly in CF patients such a rise in factor VIII [16] or hyperhomocysteinaemia.…”

“…Recently, a thrombophilic tendency has been reported in the CF population [14][15][16][17][18], the significance of which remains unclear. Some authors have thus recommended including thrombophilia screening prior to catheter insertion for all CF patients [14,15].…”

Thromboelastogram as a Tool to Predict Hypercoagulability in Children With Cystic Fibrosis

“…Other studies have reported CF-associated changes in the hemostatic system in a procoagulant direction based on the measurements of isolated components of the coagulation pathways. 13,14,22 –24 No previous study has examined plasma coagulation by TEG in CF; however, we have previously demonstrated that CF displays hypercoagulability in whole blood by TEG in accordance with the results of this study. The results of the present study yielded significant hypercoagulability in children with CF, as detected by TEG.…”

“…Cystic fibrosis is a chronic inflammatory condition with a purported increased incidence of thrombophilia, although thromboembolic disease is rarely reported. 13,14 It has been reported that patients with CF have cytokine dysregulation and an excessive host inflammatory response. This excessive host inflammatory response exposes the vascular endothelium to unchecked chronic inflammation.…”

A woman with cystic fibrosis, severe hypoxaemia, an atrial thrombus and a patent foramen ovale: a case report

“…Progressive hypoxaemia occurs as a result of pulmonary sepsis and associated obstruction of the airways [1]. Despite being a chronic inflammatory condition with a purported increased incidence of thrombophilia [2,3], thromboembolic disease is rarely reported except in association with totally implantable venous access devices (TIVADs) [4]-[8] which are often inserted when frequent courses of intravenous antibiotics are required.…”