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Case Reports
. 2017 Mar 30;5(5):664-670.
doi: 10.1002/ccr3.890. eCollection 2017 May.

Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature

Eva Jennes  1 , Dorothee Guggenberger  2 , Rainer Zotz  3 , Lora Thompson  1 , Tim H Brümmendorf  1 , Steffen Koschmieder  1 , Edgar Jost  1
Affiliations
Case Reports

Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature

Eva Jennes et al. Clin Case Rep. .

Abstract

Acquired von Willebrand syndrome may be related to plasma cell dyscrasia and can cause severe bleeding complications. Treatment, for example, with intravenous immunoglobulins may be indicated in selected cases. Physicians treating plasma cell dyscrasia have to be aware of bleeding complications in these patients, and clarification is necessary.

Keywords: Acquired von Willebrand syndrome; bleeding; intravenous immunoglobulins; monoclonal gammopathy of undetermined significance; multimer analysis.

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Figures

Figure 1
Figure 1
Multimer analysis of the patient showing a type 2A/3 pattern with a globally decreased VWF and with a very low proportion of high molecular weight multimers. The figure shows the multimer analysis before and after 3 days of therapy with IVIG 0.5 g/kg body weight and comparison to a normal subject (NPI) (gel 1.2%).
Figure 2
Figure 2
The figure shows the course of VWF:Ag, VWF:RCo, and PFA‐100 closure time. The time of treatment with dexamethasone is marked with a black box, the infusion of IVIG is marked with a gray box, and the day of the surgical intervention is marked with an arrow.
See this image and copyright information in PMC

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