Continuous-infusion von Willebrand factor concentrate is effective for the management of acquired von Willebrand disease.

Autor:	Dane KE; Department of Pharmacy and., Lindsley JP; Department of Pharmacy and., Kickler T; Division of Hematology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD., Streiff MB; Division of Hematology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD., Moliterno A; Division of Hematology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD., Yui J; Division of Hematology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD., Naik R; Division of Hematology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD., Chaturvedi S; Division of Hematology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD.
Jazyk:	angličtina
Zdroj:	Blood advances [Blood Adv] 2021 Jul 27; Vol. 5 (14), pp. 2813-2816.
DOI:	10.1182/bloodadvances.2021004843
Abstrakt:	Acquired von Willebrand disease (aVWD) is a rare disorder associated with a reduction in von Willebrand factor (VWF) activity, leading to increased bleeding risk. Monoclonal gammopathy of undetermined significance (MGUS) is the most common cause of lymphoproliferative disorder-associated aVWD and is caused by accelerated clearance of circulating VWF. Standard VWF replacement protocols for congenital VWD based on intermittent bolus dosing are typically less effective for aVWD because of antibody-mediated clearance. Intermittent bolus dosing of VWF concentrates often leads to inadequate peak response and profoundly shortened VWF half-life in aVWD. Intravenous immune globulin (IVIG) has demonstrated efficacy in aVWD; however, treatment effect is delayed up to 4 days, limiting its efficacy in acutely bleeding patients. We report the successful use of continuous-infusion VWF concentrate (with or without concomitant IVIG) in 3 patients with MGUS-associated aVWD who had demonstrated an inadequate response to bolus dosing. VWF concentrate doses required in this cohort were higher than typical doses for bleeding treatment in congenital VWD. This report illustrates that continuous-infusion VWF concentrate administration with or without intravenous immunoglobulin rapidly achieves target ristocetin cofactor activity and provides adequate hemostasis in aVWD associated with immunoglobulin G MGUS. (© 2021 by The American Society of Hematology.)
Databáze:	MEDLINE
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