2023 Volume 59 Issue 7 Pages 1110-1113
We report a case of acute appendicitis in a teenage female with a history of Type 2A von Willebrand disease (VWD) who presented to our department with right lower abdominal pain. She was diagnosed with cellulitis-associated acute appendicitis and underwent conservative therapy considering the risk of bleeding. Blood tests during that time revealed a platelet count of 511,000, a PT-INR of 1.04, an APTT of 40 s, a VWF activity of <6%, and an FVIII activity of 23%. Given the presence of a fecalith in acute appendicitis, we planned for an interval appendectomy to minimize the risk of recurrence. During the waiting period for surgery, she experienced menstruation, and we utilized a recombinant von Willebrand factor (rVWF) product, which became available in Japan in 2020. The dose of rVWF for perioperative use was determined on the basis of VWF and FVIII activities at that time. Preparations for the perioperative period were conducted in accordance with the 2021 edition of the VWD treatment guidelines. Laparoscopic appendectomy was performed using standard techniques, and there were no bleeding events during the perioperative period, ensuring a safe procedure. In this report, we present a case of appendicitis in a patient with VWD Type 2A, a qualitative abnormality of the von Willebrand factor, and describe the use of the rVWF product for interval appendectomy.
