[Isolated increased aPTT with anamnestic hemorrhagic diathesis--severe FXI deficiency]

A 47-year-old patient with several episodes of bleeding after tonsillectomy, after an excision of a sacral dermoid and after urinary tract surgery presented with a mechanical ileus and was admitted to the department of visceral surgery. Preoperative analysis revealed a prolonged activated partial thromboplastin time (aPTT) of 93 seconds (normal range: 40-60 seconds), whereas the prothrombin time and thrombin time were normal. A mixture of 1 volume patient plasma and 1 volume normal plasma gave a normal aPTT, both before and after incubation of the plasma mixture at 37 degrees C for 1 hour. The patient's history and the prolonged aPTT as the only abnormal clotting test indicated deficiency of either factor VIII, factor IX, factor XI or von Willebrand factor with consecutively diminished factor VIII. Laboratory analysis revealed a factor XI of 4% indicating severe factor XI deficiency. Laparotomy was successfully performed without any hemorrhagic complications under fresh frozen plasma substitution.