There are errors in the data presented in Tables 2 and 5. As a result, incorrect data values are also quoted in several places within the text of the manuscript.
In the last row of Table 2 on page 2041, “0.7 (0.0-6.0)” should be “6.5 (0-60).”
In lines 5 and 6 of the first full paragraph on page 2042, “median and 2-sided 95% CIs for the mean (standard deviation)” should read “mean (standard deviation) and 2-sided 95% CIs for the mean.”
Also, the data presented in Table 5 (page 2042) and Figure 3 (page 2043) are incorrect. The corrected Table 5 and Figure 3 are shown below. As a result of these errors, the mean von Willebrand factor (VWF):ristocetin cofactor activity (RCo) terminal half-life listed in the abstract on page 2038, “Results” on page 2043, and “Discussion” on page 2044 should be 22.6 hours (95% confidence interval [CI], 19.5-25.7) for recombinant VWF (rVWF) alone and 22.5 hours (95% CI, 17.4-27.6) for rVWF administered with recombinant factor VIII (rFVIII).
Pharmacokinetic data for rVWF and rVWF:rFVIII
| Mean (SD) | ||||||
|---|---|---|---|---|---|---|
| 95% CI of mean | ||||||
| 50 IU VWF:RCo/kg | 80 IU rVWF VWF:RCo/kg | |||||
| PK1 (N = 15) | PK2 (N = 13) | Type 3 VWD only | ||||
| rVWF:rFVIII (N = 16) | rVWF (N = 14) | PK1 (N = 11) | PK2 (N = 10) | |||
| T1/2 (h) | 22.5 (9.6) | 22.6 (5.3) | 19.1 (4.3) | 21.0 (5.7) | 19.1 (4.9) | 20.5 (4.7) |
| 17.4-27.6 | 19.5-25.7 | 16.7-21.5 | 17.6-24.5 | 15.8-22.4 | 17.1-23.9 | |
| AUC0-∞/dose ([h*U/dL]/[U VWF:RCo/kg]) | 41.7 (14.8) | 42.1 (8.3) | 36.8 (9.0) | 38.2 (9.1) | 38.4 (7.1) | 38.2 (10.3) |
| 33.8-49.6 | 37.3-46.9 | 31.8-41.8 | 32.8-43.7 | 33.6-43.2 | 30.8-45.5 | |
| Cmax (U/dL) | 95.9 (27.6) | 93.6 (21.0) | 155.7 (31.6) | 150.2 (34.5) | 161.8 (32.3) | 152.5 (30.6) |
| 81.2-110.6 | 81.5-105.8 | 138.2-173.2 | 129.4-171.1 | 140.1-183.5 | 130.6-174.4 | |
| IR at Cmax ([U/dL]/[U VWF:RCo/kg]) | 1.9 (0.5) | 1.9 (0.4) | 2.0 (0.4) | 1.9 (0.4) | 2.0 (0.4) | 1.9 (0.4) |
| 1.6-2.2 | 1.6-2.1 | 1.7-2.2 | 1.6-2.1 | 1.8-2.3 | 1.6-2.2 | |
| CL (dL/kg/h) | 0.027 (0.010) | 0.025 (0.005) | 0.029 (0.009) | 0.028 (0.007) | 0.027 (0.007) | 0.028 (0.008) |
| 0.022-0.032 | 0.022-0.028 | 0.024-0.034 | 0.023-0.032 | 0.023-0.032 | 0.022-0.034 | |
| Vss (dL/kg) | 0.79 (0.27) | 0.77 (0.15) | 0.75 (0.22) | 0.79 (0.26) | 0.71 (0.21) | 0.76 (0.17) |
| 0.64-0.93 | 0.68-0.86 | 0.62-0.87 | 0.63-0.95 | 0.56-0.85 | 0.64-0.88 | |
| MRT (h) | 32.2 (14.5) | 31.9 (7.5) | 26.1 (6.0) | 29.3 (9.8) | 26.3 (6.9) | 28.2 (7.6) |
| 24.4-39.9 | 27.6-36.3 | 22.8-29.5 | 23.4-35.3 | 21.7-30.9 | 22.7-33.6 | |
| Mean (SD) | ||||||
|---|---|---|---|---|---|---|
| 95% CI of mean | ||||||
| 50 IU VWF:RCo/kg | 80 IU rVWF VWF:RCo/kg | |||||
| PK1 (N = 15) | PK2 (N = 13) | Type 3 VWD only | ||||
| rVWF:rFVIII (N = 16) | rVWF (N = 14) | PK1 (N = 11) | PK2 (N = 10) | |||
| T1/2 (h) | 22.5 (9.6) | 22.6 (5.3) | 19.1 (4.3) | 21.0 (5.7) | 19.1 (4.9) | 20.5 (4.7) |
| 17.4-27.6 | 19.5-25.7 | 16.7-21.5 | 17.6-24.5 | 15.8-22.4 | 17.1-23.9 | |
| AUC0-∞/dose ([h*U/dL]/[U VWF:RCo/kg]) | 41.7 (14.8) | 42.1 (8.3) | 36.8 (9.0) | 38.2 (9.1) | 38.4 (7.1) | 38.2 (10.3) |
| 33.8-49.6 | 37.3-46.9 | 31.8-41.8 | 32.8-43.7 | 33.6-43.2 | 30.8-45.5 | |
| Cmax (U/dL) | 95.9 (27.6) | 93.6 (21.0) | 155.7 (31.6) | 150.2 (34.5) | 161.8 (32.3) | 152.5 (30.6) |
| 81.2-110.6 | 81.5-105.8 | 138.2-173.2 | 129.4-171.1 | 140.1-183.5 | 130.6-174.4 | |
| IR at Cmax ([U/dL]/[U VWF:RCo/kg]) | 1.9 (0.5) | 1.9 (0.4) | 2.0 (0.4) | 1.9 (0.4) | 2.0 (0.4) | 1.9 (0.4) |
| 1.6-2.2 | 1.6-2.1 | 1.7-2.2 | 1.6-2.1 | 1.8-2.3 | 1.6-2.2 | |
| CL (dL/kg/h) | 0.027 (0.010) | 0.025 (0.005) | 0.029 (0.009) | 0.028 (0.007) | 0.027 (0.007) | 0.028 (0.008) |
| 0.022-0.032 | 0.022-0.028 | 0.024-0.034 | 0.023-0.032 | 0.023-0.032 | 0.022-0.034 | |
| Vss (dL/kg) | 0.79 (0.27) | 0.77 (0.15) | 0.75 (0.22) | 0.79 (0.26) | 0.71 (0.21) | 0.76 (0.17) |
| 0.64-0.93 | 0.68-0.86 | 0.62-0.87 | 0.63-0.95 | 0.56-0.85 | 0.64-0.88 | |
| MRT (h) | 32.2 (14.5) | 31.9 (7.5) | 26.1 (6.0) | 29.3 (9.8) | 26.3 (6.9) | 28.2 (7.6) |
| 24.4-39.9 | 27.6-36.3 | 22.8-29.5 | 23.4-35.3 | 21.7-30.9 | 22.7-33.6 | |
CL, clearance; Cmax, maximum concentration; MRT, mean residence time; PK1, initial PK assessment; PK2, PK assessment 6 mo after initial PK; SD, standard deviation.
VWF:RCo of rVWF. Median, IQR, and range of VWF:RCo after infusion of 50 IU VWF:RCo/kg rVWF alone and together with rFVIII.
VWF:RCo of rVWF. Median, IQR, and range of VWF:RCo after infusion of 50 IU VWF:RCo/kg rVWF alone and together with rFVIII.
Under “PK” on page 2043, the ratio between the geometric mean AUC0-∞/dose of VWF:RCo for rVWF alone vs rVWF administered with rFVIII should be “1.02; 90% CI, 0.93-1.11.” In the same section, as well as in “Discussion” on page 2044, the incremental recovery (IR) should be 1.9 U/dL VWF:RCo/kg for rVWF with or without rFVIII.
These errors have been corrected in the online version of the article.
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