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Heterogeneity of type I von Willebrand disease: evidence for a subgroup with an abnormal von Willebrand factor
| Content Provider | Scilit |
|---|---|
| Author | Mannucci, Pm Lombardi, R. Bader, R. Vianello, L. Federici, Ab Solinas, S. Mazzucconi, Mg Mariani, G. |
| Copyright Year | 1985 |
| Description | Type I von Willebrand disease (vWD) is characterized by equally low plasma concentrations of von Willebrand factor antigen (vWF:Ag) and ristocetin cofactor (RiCof) and by the presence of all vWF multimers in sodium dodecyl sulfate (SDS)-agarose gel electrophoresis. For 17 patients (13 kindreds) diagnosed with these criteria, we have studied the platelet contents of vWF:Ag and RiCof and the changes of these in plasma after DDAVP infusion. Platelet vWF:Ag and RiCof were normal in four kindreds (called “platelet normal” subgroup); following 1-deamino- 8-D-arginine vasopressin; plasma vWF:Ag, RiCof and the bleeding time (BT) became normal. In six kindreds, platelet vWF:Ag and RiCof were equally low (platelet low); after DDAVP, plasma vWF:Ag and RiCof remained low, and the BT was prolonged. In three additional kindreds, platelets contained normal concentrations of vWF:Ag, but RiCof was very low (platelet discordant); even though a complete set of multimers was found in plasma and platelets, there was a relatively small amount of large multimers. After DDAVP, plasma vWF:Ag became normal, but RiCof remained low and the BT was very prolonged. These findings demonstrated that there can be an abnormal vWF (RiCof less than vWF:Ag) even in type I vWD, coexisting with a complete set of vWF multimers (platelet discordant); that the abnormal vWF can be shown more clearly in platelets than in plasma or else in plasma after DDAVP infusion; and that DDAVP normalizes the BT only in those patients with normal platelet levels of both vWF:Ag and RiCof (platelet normal). |
| Related Links | https://ashpublications.org/blood/article-pdf/66/4/796/593587/796.pdf |
| Ending Page | 802 |
| Page Count | 7 |
| Starting Page | 796 |
| DOI | 10.1182/blood.v66.4.796.796 |
| Journal | Blood |
| Issue Number | 4 |
| Volume Number | 66 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 1985-10-01 |
| Access Restriction | Open |
| Subject Keyword | Hematology Sodium Antigen Abnormal Ddavp Von Vwd Vwf Multimers Willebrand Journal: Blood (Vol- 75, Issue- 4) |
| Content Type | Text |
| Resource Type | Article |
