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Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches.
Blood. 1998 Oct 15; 92(8):2707-11.Blood

Abstract

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 IgGkappa (3) or lambda (5) MGUS and 2 IgM-kappa 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.

Authors+Show Affiliations

Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, University of Milano and Department of Hematology, S. Bortolo Hospital, Vicenza, Italy. Augusto.Federici@unimi.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Comparative Study
Controlled Clinical Trial
Journal Article
Multicenter Study

Language

eng

PubMed ID

9763553

Citation

Federici, A B., et al. "Treatment of Acquired Von Willebrand Syndrome in Patients With Monoclonal Gammopathy of Uncertain Significance: Comparison of Three Different Therapeutic Approaches." Blood, vol. 92, no. 8, 1998, pp. 2707-11.
Federici AB, Stabile F, Castaman G, et al. Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood. 1998;92(8):2707-11.
Federici, A. B., Stabile, F., Castaman, G., Canciani, M. T., & Mannucci, P. M. (1998). Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood, 92(8), 2707-11.
Federici AB, et al. Treatment of Acquired Von Willebrand Syndrome in Patients With Monoclonal Gammopathy of Uncertain Significance: Comparison of Three Different Therapeutic Approaches. Blood. 1998 Oct 15;92(8):2707-11. PubMed PMID: 9763553.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. AU - Federici,A B, AU - Stabile,F, AU - Castaman,G, AU - Canciani,M T, AU - Mannucci,P M, PY - 1998/10/9/pubmed PY - 1998/10/9/medline PY - 1998/10/9/entrez SP - 2707 EP - 11 JF - Blood JO - Blood VL - 92 IS - 8 N2 - 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 IgGkappa (3) or lambda (5) MGUS and 2 IgM-kappa 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. SN - 0006-4971 UR - https://www.unboundmedicine.com/medline/citation/9763553/Treatment_of_acquired_von_Willebrand_syndrome_in_patients_with_monoclonal_gammopathy_of_uncertain_significance:_comparison_of_three_different_therapeutic_approaches_ L2 - http://www.diseaseinfosearch.org/result/9536 DB - PRIME DP - Unbound Medicine ER -