PB1014 - Acquired Von Willebrand Syndrome associated with Renal Cell Carcinoma: A case report.

Background: Acquired Von Willebrand syndrome (AVWS) is a rare bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding. Most cases of AVWS have been associated with lymphoproliferative disorders (48%), cardiovascular disease (21%), and myeloproliferative diseases (15%). It has also been described in association with non-haematologic neoplasms such as Wilms' tumour, Ewing's sarcoma, and peripheral neuroectodermal tumours, and more rarely in bladder and prostate cancers. Mechanisms proposed for AVWS include specific or nonspecific antibodies to VWF, aberrant tumour expression of GP1b or GP IIb/IIIa receptors resulting in absorption of VWF, increased proteolytic degradation of VWF, or loss of high-volume VWF multimers under shear stress. Diagnosis requires the absence of personal or family history of bleeding with findings typical of Von Willebrand deficiency (VWD; reduced VWF antigen, VWF:RCo, and VWF:RCo/ antigen ratio).

Aims: To increase awareness of one rare disease association with AVWS

Methods: A forty-eight-year-old female presented with complaints of prolonged bleeding following partial nephrectomy for clear cell renal cell carcinoma (RCC), requiring red cell transfusion. Over the past 1 year, there is history of prolonged bleeding from minor wounds, ecchymoses, menorrhagia, and one episode of bleeding from minor wound requiring surgical haemostasis. In view of bleeding manifestations, she was referred for a complete coagulation work-up.

Results: ISTH BAT score: 14
Skin Bleeding Time: >15 minutes
Blood Group: "O"
Details of the complete coagulation work-up are given in Table 1.

Conclusion(s): To the best of our knowledge, AVWS secondary to a clear cell RCC is extremely rare with one case reported in literature. As the laboratory phenotype of our case was type 3, the aetiology could be autoantibody related. However, the confirmatory test by VWFpp (propeptide) was not performed. We iterate the fact that AVWS should be in the list of a clinician's differentials when patients develop bleeding symptoms in the setting of renal tumours.