Factor XI Deficiency Clinical Presentation

Updated: Jun 15, 2022
  • Author: Jamie E Siegel, MD; Chief Editor: Srikanth Nagalla, MD, MS, FACP  more...
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Bleeding in persons with factor XI (FXI) deficiency occurs with dental extractions, trauma, or surgery. The FXI level does not correlate with or act as a predictor of bleeding risk. [16] Within individual patients and their family, highly variable and unpredictable bleeding patterns occur. Bleeding can be immediate or delayed.

Considerations in the history are as follows:

  • Circumcision may be the first manifestation of this bleeding disorder, but a negative history does not exclude FXI deficiency
  • Bleeding with dental extractions is a common manifestation
  • Menorrhagia has been reported in as many as 59% of women with FXI deficiency; in one study of women with menorrhagia, 4% were found to have FXI deficiency. [2]
  • An inconsistent history of bleeding in the same patient may be observed with FXI deficiency.
  • FXI deficiency in an asymptomatic patient may be identified only by a prolonged activated partial thromboplastin time (aPTT) on routine preoperative testing.
  • Family history may reflect an autosomal dominant or recessive pattern of inheritance.

Physical Examination

Physical manifestations of FXI deficiency are rare. Bruising and petechiae usually are not observed with this coagulation disorder. No chronic joint abnormalities occur. After a surgical procedure, if a patient has remained undiagnosed and untreated, a significant hematoma may occur in the area of surgery.