Central venous catheter-associated thrombosis in severe haemophilia

Significant subclavian vein thromboses associated with indwelling fully implanted (port-a-cath) devices are described in two boys with severe haemophilia A and factor VIII inhibitors. Investigations were prompted by prominent chest wall veins in one case, whereas the thrombosis was a chance finding in the other case during investigation of mechanical dislocation of the catheter tubing. Extensive collateral venous circulations were demonstrated by venography in both instances indicating that the thrombus had been present for some time. Possible contributing factors to the thromboses included desensitization therapy (both patients), high-dose FEIBA (in one patient) and use of lower doses of heparin for line flush than that recommended by some authors. Neither patient had a familial or non-familial predisposition to thrombosis.