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von Willebrand disease "Vicenza" with larger-than-normal (supranormal) von Willebrand factor multimers.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Mannucci, Pier Mannuccio Lombardi, R. Castaman, Giancarlo Dent, Judith A. Lattuada, Antonella Rodeghiero, Francesco Zimmerman, Theodore S. |
| Copyright Year | 1988 |
| Abstract | When normal volunteers or patients with type I von Willebrand disease (VWD) are given desmopressin (DDAVP), a set of larger-than-normal (supranormal) von Willebrand factor (VWF) multimers, similar to those present in VWF-containing cells such as platelets megakaryocytes and endothelial cells, appear transiently in postinfusion plasma. In two kindreds with mild lifelong bleeding symptoms transmitted as an autosomal dominant trait, all ten symptomatic members (but none of the five asymptomatic members) had a supranormal multimeric structure for plasma VWF, apparently identical to that seen for postdesmopressin normal plasma. Plasma factor VIII coagulant activity (VIII:C), VWF antigen (VWF:Ag), ristocetin-induced platelet agglutination, and ristocetin cofactor (RiCof) activity were low. Platelet VWF:Ag and RiCof levels (tested for three patients only) were normal. Bleeding times were normal or slightly prolonged. The patients' platelet multimeric structure was the same as that for normal platelets. After desmopressin infusion the plasma VWF multimeric structure remained supranormal as for preinfusion plasma, with VIII:C VWF:Ag and RiCof increasing markedly over baseline values and disappearing at a normal rate. Examination of the VWF subunit composition from three of these patients indicated that proteolytic processing of their VWF did not differ from normal. This study describes the first variant of VWD with a supranormal multimeric structure. |
| Starting Page | 65 |
| Ending Page | 70 |
| Page Count | 6 |
| File Format | PDF HTM / HTML |
| DOI | 10.1182/blood.v71.1.65.65 |
| PubMed reference number | 3257148 |
| Journal | Medline |
| Volume Number | 71 |
| Issue Number | 1 |
| Alternate Webpage(s) | http://www.bloodjournal.org/content/bloodjournal/71/1/65.full.pdf?sso-checked=true |
| Alternate Webpage(s) | https://doi.org/10.1182/blood.v71.1.65.65 |
| Journal | Blood |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |
