Acquired von Willebrand syndrome in a patient with rectal adenocarcinoma: a case report and review of the literature

BACKGROUND: Acquired von Willebrand syndrome is a rare and often underdiagnosed entity, secondary to different pathologies, often of autoimmune or paraneoplastic etiology. CASE PRESENTATION: Here, we report the first case of acquired von Willebrand syndrome secondary to rectal adenocarcinoma in a 62-year-old white patient with a negative history of a hemorrhagic diathesis. The patient received chemo-radiotherapy with tumor mass decrease and subsequent surgical removal. A pre-surgery workup identified isolated prolonged aPTT that led to coagulation factor analysis. Following prophylactic von Willebrand factor substitution, the surgery was performed without hemorrhagic complications. After treatment of the oncological disease, we observed spontaneous resolution of von Willebrand syndrome. CONCLUSIONS: It is important to recognize acquired von Willebrand syndrome because it can put the patient at a high risk of bleeding complications. Our case reports, for the first time, the presence of this syndrome secondary related to rectal adenocarcinoma. We also reviewed a few cases of acquired von Willebrand syndrome secondary to underlying solid tumors described in the literature.