Acquired von Willebrand Syndrome Associated With Monoclonal Gammopathy: A Single-Center Study of 36 Patients

摘要

In this single-center retrospective study, we evaluated the accuracy of laboratory tests in diagnosing acquired von Willebrand syndrome associated with lymphoproliferative disorders in 36 consecutive patients diagnosed at the University Hospital of Nantes, France. We also compared hemostatic treatments in the following groups: 21 patients with Waldenstr?m macroglobulinemia (WM), 14 with monoclonal gammopathy of undetermined significance (MGUS) (10 with IgG-MGUS and 4 with IgM-MGUS), and 1 with IgA multiple myeloma (IgA-MM). The diagnosis was made in 18 (50%) patients during systematic screening, in 6 (17%) during active mild hemorrhage, and in 12 (33%) during an active, severe bleed. Of the laboratory tests studied, only closure times measured on the Platelet Function Analyzer (PFA)-100 device reliably diagnosed the hemostatic problem. There was no relationship between the factor VIII activity (FVIII:C) or von Willebrand factor activity (VWF:RCo) levels and the previous history of hemorrhage described by patients. We studied hemostatic treatment in most patients: IgG-MGUS patients responded well to high-dose intravenous immunoglobulin (IVIg) infusions (1 g/kg per d), although patients with IgM-MGUS did not. Desmopressin infusions were effective in 3 patients with IgG-MGUS and 2 patients with IgM-MGUS when the baseline values were above 10 IU/dL, but levels soon returned to the baseline. The 7 WM patients had a good response to desmopressin. These results confirm the efficacy of IVIg in IgG-MGUS patients and the prominent role of closure time in the diagnosis of acquired von Willebrand syndrome. Abbreviations: AVWS = acquired von Willebrand syndrome, CT-ADP = closure time test with ADP cartridge, CT-EPI = closure time test with epinephrine cartridge, DDAVP = desmopressin (1-desamino-8-D-arginine vasopressin), FVIII:C = factor VIII activity, ISTH = International Society on Thrombosis and Haemostasis, IVIg = intravenous immunoglobulin, MG = monoclonal gammopathy, MGUS= monoclonal gammopathy of undetermined significance, MM = multiple myeloma, PFA = Platelet Function Analyzer, VWF = von Willebrand factor, VWF:Ag = Von Willebrand factor antigen, VWF:RCo = von Willebrand factor activity, WM = Waldenstr?m macroglobulinemia.