PB0903 - Thromboembolic events in patients with inherited bleeding disorders – case series from a single institution

Background: Inherited deficiencies of blood coagulation factors are usually associated with life long bleeding tendency. But, there are reports of thrombotic events both arterial and venous.

Aims: This study aimed to assess the frequency and causes of these complications in adult patients diagnosed and treated in the Wroclaw Medical University, Poland

Methods: The retrospective analysis was performed in 231 patients with bleeding disorders treated in our center in the last 5 years. Thrombosis was defined as any thrombotic event recorded in the documentation. The location of the clot and possible causes were investigated.

Results: As shown in Table 1 thrombotic events occurred in 10 persons (5 females and 5 males, aged from 31 to 72) out of 231 patients with inherited bleeding disorders (2.3 %). There were seven venous thrombosis and three arterial thrombosis. The antiphospholipid antibodies were found in two patients, the heterozygous FVLeiden mutation in one, and the other one had cancer. Out of three patients aged > 65 years two had arterial thrombosis and one venous thrombosis. In all 10 patients thrombosis were not associated with use of long-central venous catheters or with intensive repalcement therapy.

Conclusion(s): Deficiencies of coagulation factors do not always protect from the risk of thrombosis. Thus, in patients with history of thrombosis, it may be helpful to perform a screening for some hereditary (e.g. factor V Leiden mutation, prothrombin gene mutation) and acquired (e.g. lupus anticoagulant) risk for thrombosis.