Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling.

Background: The acute management of acquired von Willebrand syndrome (AVWS) is aimed at achieving hemostasis with von Willebrand factor replacement, counteracting the pathologic antibodies with intravenous immunoglobulin (IVIG), and supportive care with blood transfusions. However, strategies for the long-term management of AVWS are not described, resulting in persistent use of these acute strategies to achieve hemostasis via high utilization of blood products. Herein, we provide an updated review of the use of IVIG and rituximab for AVWS and present rituximab maintenance as an effective and durable strategy for the management of these patients.

Case Report: We report the successful treatment of AVWS with anti-CD20 monoclonal antibody therapy (375 mg/m2 rituximab as four weekly doses followed by 375 mg/m2 every 90 days) in a patient with concurrent monoclonal B-cell lymphocytosis allowing for the early discontinuation of blood product support after only 2 g/kg IVIG achieved acute hemostasis control.

Results: This is the first documentation of the successful long-term management of AVWS without prolonged blood product or IVIG support. This result contrasts sharply to previously reported rituximab strategies that were deemed ineffective in AVWS.

Conclusion: A maintenance regimen of rituximab may be an effective long-term management strategy for AVWS associated with lymphoproliferative disorders, which may minimize the use of blood products and IVIG.