Characteristics of response to desmopressin according to the presence of abnormal or normal basal multimeric pattern and presence of mutation
| . | Complete response, n = 64 . | Partial response, n = 10 . | No response, n = 3 . | P* . |
|---|---|---|---|---|
| Male/female | 25/39 | 3/7 | 1/2 | .88 |
| Median age, y (range) | 38 (6-67) | 41 (13-62) | 49 (21-75) | .61 |
| Blood group O (%) | 36 (56.2) | 7 (70.0) | 1 (33.3) | .55 |
| Median VWF:RCo, IU/dL (interquartile range) | 44.5 (31) | 7 (8) | 3 (13) | .001 |
| Median VWF:Ag, IU/dL (interquartile range) | 44 (34) | 15 (7) | 17 (7) | .004 |
| Median FVIII:C, IU/dL (interquartile range) | 53 (54) | 23 (6) | 28 (44) | .007 |
| Mean VWF:RCo/VWF:Ag ratio (interquartile range) | 0.94 (0.39) | 0.52 (0.63) | 0.22 (0.82) | .005 |
| Groups | .002 | |||
| 1: Abnormal VWF multimers (%) | 22 (64.7) | 9 (26.4) | 3 (8.8) | — |
| 2: Normal VWF multimers and a mutation (%) | 26 (96.3) | 1 (3.7) | 0 (0) | — |
| 3: Normal VWF multimers and no mutation (%) | 16 (100) | 0 (0) | 0 (0) | — |
| Identified mutations, no. | .70 | |||
| None | 18 | 1 | 0 | — |
| One | 37 | 8 | 2 | — |
| Two | 8 | 1 | 1 | — |
| Three | 1 | 0 | 0 | — |
| . | Complete response, n = 64 . | Partial response, n = 10 . | No response, n = 3 . | P* . |
|---|---|---|---|---|
| Male/female | 25/39 | 3/7 | 1/2 | .88 |
| Median age, y (range) | 38 (6-67) | 41 (13-62) | 49 (21-75) | .61 |
| Blood group O (%) | 36 (56.2) | 7 (70.0) | 1 (33.3) | .55 |
| Median VWF:RCo, IU/dL (interquartile range) | 44.5 (31) | 7 (8) | 3 (13) | .001 |
| Median VWF:Ag, IU/dL (interquartile range) | 44 (34) | 15 (7) | 17 (7) | .004 |
| Median FVIII:C, IU/dL (interquartile range) | 53 (54) | 23 (6) | 28 (44) | .007 |
| Mean VWF:RCo/VWF:Ag ratio (interquartile range) | 0.94 (0.39) | 0.52 (0.63) | 0.22 (0.82) | .005 |
| Groups | .002 | |||
| 1: Abnormal VWF multimers (%) | 22 (64.7) | 9 (26.4) | 3 (8.8) | — |
| 2: Normal VWF multimers and a mutation (%) | 26 (96.3) | 1 (3.7) | 0 (0) | — |
| 3: Normal VWF multimers and no mutation (%) | 16 (100) | 0 (0) | 0 (0) | — |
| Identified mutations, no. | .70 | |||
| None | 18 | 1 | 0 | — |
| One | 37 | 8 | 2 | — |
| Two | 8 | 1 | 1 | — |
| Three | 1 | 0 | 0 | — |
— indicates not applicable.
Tests difference among the 3 groups.