CASE REPORT OF RECURRENT ACQUIRED VON WILLEBRAND FACTOR DEFICIENCY WITH HISTORY OF SEVERAL ENDOSCOPY EXAMINATIONS AND SMALL BOWEL RESECTIONS

Aims:

To report a case of recurrent acquired von Willebrand factor (vWF) deficiency.

Methods:

The patient has undergone several capsule endoscopy (CE) examinations and small bowel resections.

Results:

A 59 years old man with overt gastrointestinal bleeding and iron-deficiency anemia visited our referral center with a history of several episodes of melena during 2010 – 2015. In 2012, he was operated upon and two short segments of small intestine were resected, surgical sample confirmed a prior CE diagnosis of angiodysplasia. After operation, gastrointestinal bleeding stopped for 2 years. In 2015 the patient experienced another episode of melena. Upper gastrointestinal endoscopy was negative for bleeding, lower gastrointestinal endoscopy was indicative of severe small intestinal bleeding. Subsequent CE examination showed one proximal and possible two distal bleeding areas with likelihood of several small intestinal angiodysplasias. During surgery two small bowel segments were again resected with histology specimens of angiodysplasia.

Noticing the history of gum bleeding, we measured vWF level in blood and found that the patient had acquired vWF deficiency. Since then small intestinal bleeding occurred several times and in spite of vWF supplementary treatment for each episode the patient required several units of blood ± plasma to stop the bleeding. In the bleeding free periods the vWF factor level remount to normal. No medical condition previously described to be associated with recurrent von Willebrand factor deficiency has been identified in our patient.

Conclusions:

In the literature recurrent von Willebrand factor deficiency is rarely described. Referral of patients to gastroenterology units with rare bleeding disorders might delay diagnosis. This case, highlights the importance of detailed medical history-taking prior to any invasive procedures. The etiology of recurrent acquired von Willebrand factor deficiency is still unknow and further investigation is warranted.