PO09 - Acquired Von Willebrand dis ease and severe gastrointestinal bleeding – haemostatic control using activated recombinant factor VII

Background: Acquired Von Willebrand disease (aVWD) is a bleeding disorder associated to increase clearance of von Willebrand Factor (VWF) and/or decrease VWF production. In aVWD secondary to lymphoproliferative disorders, immunosuppressive therapy can be addressed.

Aims: Present our clinical center approach.

Methods: We analysed clinical records, laboratory logs and blood bank registries that were deemed relevant for this clinical case.

Results: A 81 year-old female patient with a previous diagnosis of monoclonal gammopathy of undetermined significance (MGUS) had a first hospitalization in our hospital because of a lower gastrointestinal bleeding due to angiectasis. FVIII/VWF concentrates were used as haemostatic therapy and we documented a low laboratory response to FVIII/VWF concentrates, even though high doses were used with short intervals. The patient was discharged after argon plasma treatment of the colonic angiectasis. The poor response to VFW/FVII concentrates was later confirmed, with VWF:Ag, VWF:RCo and FVIII levels of 29%, 14% and 26% respectively, 1hour after VWF/FVIII concentrates administration. Two weeks later, the patient was once again admitted due to a bleeding angiectasis near the ileo-cecal valve. Due to the severity of the bleeding and the known poor response to FVIII/VWF concentrates, therapy with rFVIIa and tranexamic acid was decided. Initially dose of 90µg/Kg q2h in the first 2 days, q4h on the 3rd day, q6h on the 4th day, q8h in the 5th and 6th day. Additionally, immunosuppression was started with prednisolone 60mg/day and IV immunoglobulin 30mg/day. Discharged occurred at day 7, without any new bleeding episodes, and stable haemoglobin. We started a glucocorticoid tapering and she underwent a genetic test that did not identify any pathological mutations in the FVW gene.

Conclusion(s): In this patient, with a very poor response to FVIII/VWF concentrates, rFVIIa was effective in bleeding control. After excluding other causes, MGUS was the likely cause of aWVD and, therefore, immunosuppressive therapy was started.