Table 1.

DDAVP response by bleeding time and FVIII/VWF activities before and 2 hours after infusion, in patients with different types of von Willebrand disease divided by phenotypes and gene mutations

No. responsive patients of total
FVIII:C, U/dL
VWF:RCo, U/dL
Bleeding time, min
VWF:RCo/Ag ratio
FVIII:C/VWFAg ratio
VWD types
Before
After 2 h
Before
After 2 h
Before
After 2 h
Before
After 2 h
Before
After 2 h
All type 1  7 of 26   39 ± 24   130 ± 64   19 ± 20   59 ± 58   24 ± 10   11 ± 6   0.8 ± 0.4   0.9 ± 0.3   2.3 ± 0.3   2.3 ± 0.2  
    VWF:RCo/Ag greater than 0.6   6 of 18   44 ± 28   138 ± 72   24 ± 22   77 ± 62   23 ± 9   11 ± 7   0.9 ± 0.2   1.0 ± 0.3   2.7 ± 0.3   2.6 ± 0.2  
    VWF:RCo/Ag less than 0.6   1 of 8   28 ± 5*  110 ± 38   7 ± 1*  19 ± 10*  18 ± 12   9 ± 5   0.5 ± 0.3*  0.7 ± 0.2   1.9 ± 0.2   1.7 ± 0.2  
All type 2A  1 of 15   40 ± 14   135 ± 56   11 ± 9*  29 ± 20*  28 ± 5   23 ± 7*  0.3 ± 0.2*  0.2 ± 0.1*  1.4 ± 0.8*  1.4 ± 0.7* 
    G550R   0 of 1   35   277*  <6*  <6*  >30*  >30*  0.1*  0.1*  0.6*  3.2  
    C1272G   0 of 1   33   110   <6*  16*  >30*  >30*  0.3*  0.2*  1.6*  1.3* 
    S1506L   0 of 5   36 ± 12   91 ± 18*  12 ± 13   19 ± 9*  >30*  26 ± 8*  0.4 ± 0.2*  0.3 ± 0.2*  1.7 ± 0.8*  1.0 ± 0.2* 
    R1597W   1 of 3   45 ± 5   144 ± 9   10 ± 2   39 ± 9*  24 ± 11   18 ± 10*  0.3 ± 0.1*  0.3 ± 0.1*  1.4 ± 0.2*  1.3 ± 0.1* 
    I1628T   0 of 3   45 ± 8   149 ± 9   17 ± 5   38 ± 12*  27 ± 4   17 ± 6*  0.4 ± 0.2*  0.3 ± 0.2*  0.9 ± 0.3*  1.1 ± 0.2* 
    G1629R   0 of 1   61   203*  30   60   >30*  20*  0.3*  0.3*  0.6*  0-9* 
    V1665E   0 of 1   27   102*  <6*  10*  >30*  >30*  0.2*  0.1*  1.2*  1.0* 
All type 2M  3 of 21   37 ± 15   113 ± 41*  12 ± 6   39 ± 14*  17 ± 7   12 ± 6   0.5 ± 0.2*  0.7 ± 0.3   2.1 ± 1.2   1.9 ± 0.9  
    Vicenza, R1205H   0 of 1   4*  21*  <6*  18*  13   7   0.5*  0.6*  0.3*  0.7* 
    R1315C   0 of 3   39 ± 9   92 ± 11*  7 ± 2*  25 ± 3*  20 ± 0   12 ± 6   0.3 ± 0.1*  0.6 ± 0.2   2.0 ± 0.7   2.1 ± 0.5  
    R1374C   1 of 9   34 ± 3   117 ± 24   16 ± 11   42 ± 16   21 ± 5   15 ± 7   0.5 ± 0.3*  0.5 ± 0.3   1.9 ± 0.8   2.1 ± 0.3  
    R1374H   1 of 2   31-44   100-132   13-38   12-39   16-15   11-10   0.6-0.5   0.4-0.5   1.3-1.6   0.9*-1.8  
    undefined   1 of 6   45 ± 12   133 ± 18   10 ± 5   44 ± 6   12 ± 4*  9 ± 3   0.5 ± 0.3*  0.6 ± 0.2   2.5 ± 1.2   1.9 ± 0.7  
Type 2N  3 of 4   16 ± 7*  106 ± 60*  40 ± 13*  153 ± 72*  7 ± 3*  6 ± 2*  0.7 ± 0.1   0.9 ± 0.1   0.3 ± 0.2*  0.6 ± 0.3* 
    R854Q + R854Q   1 of 1   24   165   52*  202*  5*  5*  0.7   1.0   0.4*  0.9* 
    R854Q + null   1 of 1   13*  115   26   98*  11*  8   0.8   0.8   0.3*  0.8* 
    R854Q + C1060R   1 of 1   18*  121   48*  227*  6*  6*  0.7   0.8   0.2*  0.6* 
    C1060R + null
 		 0 of 1
 		 7*
 		 23*
 		 29
 		 85*
 		 5*
 		 4*
 		 0.7
 		 0.8
 		 0.1*
 		 0.2*
 
No. responsive patients of total
FVIII:C, U/dL
VWF:RCo, U/dL
Bleeding time, min
VWF:RCo/Ag ratio
FVIII:C/VWFAg ratio
VWD types
Before
After 2 h
Before
After 2 h
Before
After 2 h
Before
After 2 h
Before
After 2 h
All type 1  7 of 26   39 ± 24   130 ± 64   19 ± 20   59 ± 58   24 ± 10   11 ± 6   0.8 ± 0.4   0.9 ± 0.3   2.3 ± 0.3   2.3 ± 0.2  
    VWF:RCo/Ag greater than 0.6   6 of 18   44 ± 28   138 ± 72   24 ± 22   77 ± 62   23 ± 9   11 ± 7   0.9 ± 0.2   1.0 ± 0.3   2.7 ± 0.3   2.6 ± 0.2  
    VWF:RCo/Ag less than 0.6   1 of 8   28 ± 5*  110 ± 38   7 ± 1*  19 ± 10*  18 ± 12   9 ± 5   0.5 ± 0.3*  0.7 ± 0.2   1.9 ± 0.2   1.7 ± 0.2  
All type 2A  1 of 15   40 ± 14   135 ± 56   11 ± 9*  29 ± 20*  28 ± 5   23 ± 7*  0.3 ± 0.2*  0.2 ± 0.1*  1.4 ± 0.8*  1.4 ± 0.7* 
    G550R   0 of 1   35   277*  <6*  <6*  >30*  >30*  0.1*  0.1*  0.6*  3.2  
    C1272G   0 of 1   33   110   <6*  16*  >30*  >30*  0.3*  0.2*  1.6*  1.3* 
    S1506L   0 of 5   36 ± 12   91 ± 18*  12 ± 13   19 ± 9*  >30*  26 ± 8*  0.4 ± 0.2*  0.3 ± 0.2*  1.7 ± 0.8*  1.0 ± 0.2* 
    R1597W   1 of 3   45 ± 5   144 ± 9   10 ± 2   39 ± 9*  24 ± 11   18 ± 10*  0.3 ± 0.1*  0.3 ± 0.1*  1.4 ± 0.2*  1.3 ± 0.1* 
    I1628T   0 of 3   45 ± 8   149 ± 9   17 ± 5   38 ± 12*  27 ± 4   17 ± 6*  0.4 ± 0.2*  0.3 ± 0.2*  0.9 ± 0.3*  1.1 ± 0.2* 
    G1629R   0 of 1   61   203*  30   60   >30*  20*  0.3*  0.3*  0.6*  0-9* 
    V1665E   0 of 1   27   102*  <6*  10*  >30*  >30*  0.2*  0.1*  1.2*  1.0* 
All type 2M  3 of 21   37 ± 15   113 ± 41*  12 ± 6   39 ± 14*  17 ± 7   12 ± 6   0.5 ± 0.2*  0.7 ± 0.3   2.1 ± 1.2   1.9 ± 0.9  
    Vicenza, R1205H   0 of 1   4*  21*  <6*  18*  13   7   0.5*  0.6*  0.3*  0.7* 
    R1315C   0 of 3   39 ± 9   92 ± 11*  7 ± 2*  25 ± 3*  20 ± 0   12 ± 6   0.3 ± 0.1*  0.6 ± 0.2   2.0 ± 0.7   2.1 ± 0.5  
    R1374C   1 of 9   34 ± 3   117 ± 24   16 ± 11   42 ± 16   21 ± 5   15 ± 7   0.5 ± 0.3*  0.5 ± 0.3   1.9 ± 0.8   2.1 ± 0.3  
    R1374H   1 of 2   31-44   100-132   13-38   12-39   16-15   11-10   0.6-0.5   0.4-0.5   1.3-1.6   0.9*-1.8  
    undefined   1 of 6   45 ± 12   133 ± 18   10 ± 5   44 ± 6   12 ± 4*  9 ± 3   0.5 ± 0.3*  0.6 ± 0.2   2.5 ± 1.2   1.9 ± 0.7  
Type 2N  3 of 4   16 ± 7*  106 ± 60*  40 ± 13*  153 ± 72*  7 ± 3*  6 ± 2*  0.7 ± 0.1   0.9 ± 0.1   0.3 ± 0.2*  0.6 ± 0.3* 
    R854Q + R854Q   1 of 1   24   165   52*  202*  5*  5*  0.7   1.0   0.4*  0.9* 
    R854Q + null   1 of 1   13*  115   26   98*  11*  8   0.8   0.8   0.3*  0.8* 
    R854Q + C1060R   1 of 1   18*  121   48*  227*  6*  6*  0.7   0.8   0.2*  0.6* 
    C1060R + null
 		 0 of 1
 		 7*
 		 23*
 		 29
 		 85*
 		 5*
 		 4*
 		 0.7
 		 0.8
 		 0.1*
 		 0.2*
 

Numbers indicate mean ± SD or individual values for single patients.

To calculate the mean values of VWF:RCo and the VWF:RCo/Ag ratios, VWF:RCo levels below the limit of sensitivity of the assay (<6 IU/dL) were taken to 6 IU/dL.

To calculate mean and SD for bleeding times, values >30 min were taken to 30 min.

*

P<.05 compared with all patients with type 1 VWD.

View Large
Close Modal
This Feature Is Available To Subscribers Only

or Create an Account

Close Modal
Close Modal