Table 1.
Summary of patients with type 3 VWD with anti-VWF antibodies
| Patient ID/color code | Age | Sex | Race | Ethnicity | ABO | ISTH BS | VWF variant 1 | VWF variant 2 | VWF: GPIbM % inh | VWF: CB3 % inh | VWF: CB4 % inh | VWF: F8B % inh | VWF Ab class | Ab titer | Ab, A.U. |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
 051 | 11 | F | Black | Not Hisp | O | 32 | p.Trp1144∗ | p.Leu1281Cysfs∗16 | 28 | ≥94 | 27 | 43 | IgG | 800 | 135 |
 393 | 41 | F | White | Not Hisp | A | 24 | p.Met814Hisfs∗5 | p.Arg1566∗ | < | 20 | < | 57 | IgG | 400 | 47 |
 101 | 38 | M | White | Unk | O | 16 | p.Cys689∗ | p.Pro812Argfs∗31 | < | 36 | < | < | IgG | 200 | 27 |
 171 | 8 | F | Asian | Not Hisp | Unk | 4 | c.6798 + 1G>A | c.6798 + 1G>A | < | < | < | 20 | IgG | 100 | 18 |
 020 | 48 | M | White | Not Hisp | O | 10 | p.Cys331∗ | c.324-14_-4delins∗ | 11 | < | < | 15 | IgG | 100 | 12 |
 004 | 27 | F | Asian | Not Hisp | B | 6 | p.Leu970Serfs∗9 | p.Leu970Serfs∗9 | < | < | < | < | IgG | 100 | 3 |
 146 | 1 | F | White | Not Hisp | O | 6 | p.Arg575Thrfs∗16 | p.Val1546∗ | < | < | < | < | IgG | 25 | 2.5 |
 008 | 34 | F | Asian | Not Hisp | O | 10 | p.Cys665Alafs∗3 | p.Gln1113∗ | < | < | < | < | IgG | 25 | 3 |
 646 | 17 | F | White | Hispanic | B | NA | p.Leu451Argfs∗19 | p.Arg1659∗ | < | < | < | 14 | IgM | 25 | 43 |
| Patient ID/color code | Age | Sex | Race | Ethnicity | ABO | ISTH BS | VWF variant 1 | VWF variant 2 | VWF: GPIbM % inh | VWF: CB3 % inh | VWF: CB4 % inh | VWF: F8B % inh | VWF Ab class | Ab titer | Ab, A.U. |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 051 | 11 | F | Black | Not Hisp | O | 32 | p.Trp1144∗ | p.Leu1281Cysfs∗16 | 28 | ≥94 | 27 | 43 | IgG | 800 | 135 |
| 393 | 41 | F | White | Not Hisp | A | 24 | p.Met814Hisfs∗5 | p.Arg1566∗ | < | 20 | < | 57 | IgG | 400 | 47 |
| 101 | 38 | M | White | Unk | O | 16 | p.Cys689∗ | p.Pro812Argfs∗31 | < | 36 | < | < | IgG | 200 | 27 |
| 171 | 8 | F | Asian | Not Hisp | Unk | 4 | c.6798 + 1G>A | c.6798 + 1G>A | < | < | < | 20 | IgG | 100 | 18 |
| 020 | 48 | M | White | Not Hisp | O | 10 | p.Cys331∗ | c.324-14_-4delins∗ | 11 | < | < | 15 | IgG | 100 | 12 |
| 004 | 27 | F | Asian | Not Hisp | B | 6 | p.Leu970Serfs∗9 | p.Leu970Serfs∗9 | < | < | < | < | IgG | 100 | 3 |
| 146 | 1 | F | White | Not Hisp | O | 6 | p.Arg575Thrfs∗16 | p.Val1546∗ | < | < | < | < | IgG | 25 | 2.5 |
| 008 | 34 | F | Asian | Not Hisp | O | 10 | p.Cys665Alafs∗3 | p.Gln1113∗ | < | < | < | < | IgG | 25 | 3 |
| 646 | 17 | F | White | Hispanic | B | NA | p.Leu451Argfs∗19 | p.Arg1659∗ | < | < | < | 14 | IgM | 25 | 43 |
Symbol < represents value <10.
Ab, antibody; F, female; inh, inhibition; M, male; NA, not obtained; Not Hisp, not Hispanic; Unk, unknown.
∗
c.324-14_-4delins, c.324-14_324-4delinsAAGTTCAGAGTCT.