PMC4055248_01
Von Willebrand factor ristocetin cofactor (VWF:RCo) residual activity of normal pooled plasma incubated with different dilutions of plasma from the patient with acquired von Willebrand syndrome (VWS) (Patient plasma) and plasma from another patient with acquired VWS containing neutralizing anti-VWF autoantibodies (positive control).
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F1
By adaptation of the Bethesda method to VWF, the autoantibody against VWF was demonstrated to be non neutralizing (Figure 1).
Time course of haemoglobin concentration in peripheral blood of the patient with acquired von Willebrand syndrome before and after high dose immunoglobulin (Ig) infusion. Units of packed red blood cells (PRBCs) which were transfused and replacement therapy with Hemate P are reported.
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F2
IVIg, hemoglobin levels were stable at 10-11 g/dl without need of transfusion (Figure 2). IVIg had a dramatic effect on the kinetics of VWF:Ag, VWF:RCo and FVIII:C, inducing a 3- to 4-fold increase in their peak and recovery values after infusion of 2000 U of Hemate P (Figures 3, 4 and 5). Intravenous immunoglobulin also affected the levels of anti-VWF autoantibodies which progressively decreased reaching an 8-fold reduction 21 days later (Figure 6).
Kinetics of von Willebrand factor antigen (VWF:Ag) in plasma of the patient with acquired von Willebrand syndrome after replacement therapy with Hemate P. VWF:Ag levels were measured 2 days before and 2, 3 and 4 days after starting high dose intravenous immunoglobulin (Ig) infusion.
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F3
IVIg, hemoglobin levels were stable at 10-11 g/dl without need of transfusion (Figure 2). IVIg had a dramatic effect on the kinetics of VWF:Ag, VWF:RCo and FVIII:C, inducing a 3- to 4-fold increase in their peak and recovery values after infusion of 2000 U of Hemate P (Figures 3, 4 and 5). Intravenous immunoglobulin also affected the levels of anti-VWF autoantibodies which progressively decreased reaching an 8-fold reduction 21 days later (Figure 6).
Kinetics of von Willebrand factor ristocetin cofactor (VWF:RCo) in plasma of the patient with acquired von Willebrand syndrome after replacement therapy with Hemate P. VWF:RCo levels were measured 2 days before and 2, 3 and 4 days after starting high dose intravenous immunoglobulin (Ig) infusion.
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F4
IVIg, hemoglobin levels were stable at 10-11 g/dl without need of transfusion (Figure 2). IVIg had a dramatic effect on the kinetics of VWF:Ag, VWF:RCo and FVIII:C, inducing a 3- to 4-fold increase in their peak and recovery values after infusion of 2000 U of Hemate P (Figures 3, 4 and 5). Intravenous immunoglobulin also affected the levels of anti-VWF autoantibodies which progressively decreased reaching an 8-fold reduction 21 days later (Figure 6).
Kinetics of factor VIII coagulant activity (FVIII:C) in plasma of the patient with acquired von Willebrand syndrome after replacement therapy with Hemate P. Factor VIII:C levels were measured 2 days before and 2, 3 and 4 days after starting high dose intravenous immunoglobulin (Ig) infusion.
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F5
IVIg, hemoglobin levels were stable at 10-11 g/dl without need of transfusion (Figure 2). IVIg had a dramatic effect on the kinetics of VWF:Ag, VWF:RCo and FVIII:C, inducing a 3- to 4-fold increase in their peak and recovery values after infusion of 2000 U of Hemate P (Figures 3, 4 and 5). Intravenous immunoglobulin also affected the levels of anti-VWF autoantibodies which progressively decreased reaching an 8-fold reduction 21 days later (Figure 6).
Plasma levels of anti von Willebrand factor (anti-VWF) autoantibodies expressed as absorbance (Abs) at 492 nm in plasma from the patient with acquired von Willebrand syndrome before and after starting treatment with high dose intravenous immunoglobulin (Ig). The dotted line represents the upper limit of the normal range.
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PMC4055248_01_2162-3619-3-15-6.jpg
F6
IVIg, hemoglobin levels were stable at 10-11 g/dl without need of transfusion (Figure 2). IVIg had a dramatic effect on the kinetics of VWF:Ag, VWF:RCo and FVIII:C, inducing a 3- to 4-fold increase in their peak and recovery values after infusion of 2000 U of Hemate P (Figures 3, 4 and 5). Intravenous immunoglobulin also affected the levels of anti-VWF autoantibodies which progressively decreased reaching an 8-fold reduction 21 days later (Figure 6).