Characteristics of patients with suspected acquired von Willebrand syndrome (10 patients)
| Unique patient no. . | ABO blood group . | VWF:Ag, U/dL . | VWF:RCo, U/dL . | VWF:RCo/VWF:Ag ratio . | VWFpp/VWF:Ag ratio* . | Anti-VWF antibody . | IgM, g/L . | Serum viscosity, cSt . | Survival after first treatment, mo† . | Age at treatment initiation, y . | International scoring system24 . | Clinical features and treatment . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 296 | A | 64 | 21 | 0.33 | 1.84 | Absent | 42 | 3.46 | 72+ | 67 | High | Epistaxis, 4 plasma exchanges (Figure 3A) |
| 314 | O | 9 | 14 | 1.56 | 20.3 | Absent | 78 | NA | 123+ | 73 | High | Gingivorrhagia, epistaxis. 7 plasma exchanges; desmopressin before bone marrow biopsy (Figure 3B) |
| 333 | A | 47 | 45 | 0.96 | 2.06 | Absent | 36 | 2 | 76 | 77 | High | Epistaxis (Figure 3C) |
| 520 | O | 48 | 31 | 0.65 | 6.85 | NA | 47 | 3.25 | 15+ | 62 | Intermediate | Epistaxis |
| 467 | A | 69 | 43 | 0.62 | NA | NA | 39 | 3.5 | 170 | 63 | Unknown | Epistaxis; human VWF before insertion of device for plasma exchanges (7) |
| 572 | NA | 24 | 22 | 0.92 | NA | NA | 65 | 5.75 | 8+ | 42 | Low | Recombinant factor VIII before insertion of device for plasma exchange (2) |
| 577 | A | 22 | 21 | 0.95 | 4.18 | Absent | 54 | 3.72 | 6+ | 60 | Intermediate | Human VWF before bone marrow biopsy |
| 351 | O | 36 | 13 | 0.36 | 8.33 | Absent | 34 | 2.4 | 184 | 61 | Unknown | Hematoma of wrist; surgery without hemostatic therapy (Figure 3D) |
| 337 | B | 40 | 28 | 0.70 | 2.85 | Absent | 32 | 3.1 | 176 | 39 | Low | Surgery without hemostatic therapy |
| 566 | O | 38 | 32 | 0.84 | NA | NA | 50.3 | 3.18 | 8+ | 59 | Low | 3 plasma exchanges |
| Unique patient no. . | ABO blood group . | VWF:Ag, U/dL . | VWF:RCo, U/dL . | VWF:RCo/VWF:Ag ratio . | VWFpp/VWF:Ag ratio* . | Anti-VWF antibody . | IgM, g/L . | Serum viscosity, cSt . | Survival after first treatment, mo† . | Age at treatment initiation, y . | International scoring system24 . | Clinical features and treatment . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 296 | A | 64 | 21 | 0.33 | 1.84 | Absent | 42 | 3.46 | 72+ | 67 | High | Epistaxis, 4 plasma exchanges (Figure 3A) |
| 314 | O | 9 | 14 | 1.56 | 20.3 | Absent | 78 | NA | 123+ | 73 | High | Gingivorrhagia, epistaxis. 7 plasma exchanges; desmopressin before bone marrow biopsy (Figure 3B) |
| 333 | A | 47 | 45 | 0.96 | 2.06 | Absent | 36 | 2 | 76 | 77 | High | Epistaxis (Figure 3C) |
| 520 | O | 48 | 31 | 0.65 | 6.85 | NA | 47 | 3.25 | 15+ | 62 | Intermediate | Epistaxis |
| 467 | A | 69 | 43 | 0.62 | NA | NA | 39 | 3.5 | 170 | 63 | Unknown | Epistaxis; human VWF before insertion of device for plasma exchanges (7) |
| 572 | NA | 24 | 22 | 0.92 | NA | NA | 65 | 5.75 | 8+ | 42 | Low | Recombinant factor VIII before insertion of device for plasma exchange (2) |
| 577 | A | 22 | 21 | 0.95 | 4.18 | Absent | 54 | 3.72 | 6+ | 60 | Intermediate | Human VWF before bone marrow biopsy |
| 351 | O | 36 | 13 | 0.36 | 8.33 | Absent | 34 | 2.4 | 184 | 61 | Unknown | Hematoma of wrist; surgery without hemostatic therapy (Figure 3D) |
| 337 | B | 40 | 28 | 0.70 | 2.85 | Absent | 32 | 3.1 | 176 | 39 | Low | Surgery without hemostatic therapy |
| 566 | O | 38 | 32 | 0.84 | NA | NA | 50.3 | 3.18 | 8+ | 59 | Low | 3 plasma exchanges |
VWF:Ag indicates von Willebrand factor (VWF) antigen; VWF:RCo, ristocetin cofactor activity; VWFpp, propeptide; IgM, monoclonal immunoglobulin M concentration (densitometry); and NA: not available.
A VWF:pp/VWF:Ag ratio > 2.8 in case of blood group O or > 2.4 otherwise denotes an increase in the clearance of mature VWF. Cryoglobulinemia was found in patients 314, 351, 467, 572, and 577.
Censoring is denoted with +.