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Blood, Vol. 92 No. 8 (October 15), 1998:
pp. 2707-2711
Treatment of Acquired von Willebrand Syndrome in Patients With
Monoclonal Gammopathy of Uncertain Significance: Comparison of Three
Different Therapeutic Approaches
Augusto B. Federici,
Federica Stabile,
Giancarlo Castaman,
Maria
Teresa Canciani, and
Pier Mannuccio Mannucci
From the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center,
IRCCS Maggiore Hospital, University of Milano and Department of
Hematology, S. Bortolo Hospital, Vicenza, Italy.
Patients with monoclonal gammopathies of uncertain significance
(MGUS) may develop an acquired bleeding disorder similar to congenital
von Willebrand disease, called acquired von Willebrand syndrome (AvWS).
In these patients, measures to improve hemostasis are required to
prevent or treat bleeding episodes. We diagnosed 10 patients with MGUS
and AvWS: 8 had IgG (3) or  (5) MGUS and 2 IgM- MGUS. Three
therapeutic approaches were compared in them: (1) desmopressin (DDAVP),
(2) factor VIII/von Willebrand factor (FVIII/vWF) concentrate, and (3)
high-dose (1 g/kg/d for 2 days) intravenous Ig (IVIg). In patients with
IgG-MGUS, DDAVP and FVIII/vWF concentrate increased factor VIII and von
Willebrand factor in plasma, but only transiently. IVIg determined a
more sustained improvement of the laboratory abnormalities and
prevented bleeding during surgery (short-term therapy). In addition to
the standard 2-day infusion protocol, a long-term IVIg therapy was
performed in 2 patients with IgG-MGUS: repeated (every 21 days) single
infusions of IVIg did improve laboratory abnormalities and stopped
chronic gastrointestinal bleeding. On the other hand, IVIg failed to
correct laboratories abnormalities in patients with IgM-MGUS. These
comparative data obtained in a relative large and homogeneous group of
patients with AvWS and MGUS confirm that DDAVP and FVIII/vWF
concentrates improve the bleeding time (BT) and FVIII/vWF
measurements only transiently, whereas IVIg provides a sustained
treatment of AvWS associated with IgG-MGUS, but not with IgM-MGUS.
© 1998 by The American Society of Hematology.
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