Therapeutic Algorithm for Patients with Acquired Von Willebrand Syndrome and Monoclonal Gammopathy.

Abstract 4445

In contrast to congenital von Willebrand Syndrome therapy of acute bleeding in acquired von Willebrand Syndrome (AWS) with von Willebrand factor containing concentrates (F VIII/vWF) is often ineffective. In patients with IgG monoclonal antibodies therapy with intravenous immunoglobulins (IVIG) is reported to be effective whereas patients with IgM antibodies seem not to respond to IVIG.

Over the last years we diagnosed and treated several patients with AWS either prior to surgery or due to acute bleeding. About half of the patients responded adequately to IVIG as reported in the literature. One patient with no response to IVIG 4 days after the last dose received DDAVP with a significant increase in von Willebrand factor-antigen and ristocetin cofactor and normalized half life. Dental surgery could be performed without any bleeding complications under daily infusions of DDAVP. Another patient with partial response to IVIG and contraindications to DDAVP received F VIII/vWF prior to dental extraction. In contrast to former recoveries with a shortened half life of the infused concentrate (about 2 hours), half life of vWF was significantly prolonged following prior IVIG treatment. Another patient responded sufficiently to single therapy with F VIII/vWF. A further patient with suspected diagnosis of moderate hemophilia A had received DDAVP for severe epistaxis with good response and sufficient half lifes. He was transferred to our centre and we diagnosed AVW with IgM antibodies. We performed another recovery and half life with DDAVP with adequate response.

According to our experience we propose the following therapeutic algorithm: A recovery with DDAVP should be performed first line if treatment is not contraindicated. In case of insufficient half lifes and/or intended major surgery, a recovery and half life with F VIII/vWF concentrate should follow. In case of significantly reduced half life of wWF an attempt with IVIG is necessary. If response to IVIG is not adequate further treatment with DDAVP or F VIII/vWF is indicated.

As treatment with IVIG is not predictable in all patients with IgG antibodies and is very costly a therapeutic attempt with DDAVP in the first line and F VIII/vWF second line is worthwhile. In major surgery requiring prolonged replacement therapy, IVIG treatment, if effective, is less costly than treatment with FVIII/vWF.