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Platelet-type von Willebrand's disease: characterization of a new bleeding disorder
| Content Provider | Scilit |
|---|---|
| Author | Miller, Jl Castella, A. |
| Copyright Year | 1982 |
| Description | An autosomally transmitted bleeding diathesis sharing some, but not all, features previously described in von Willebrand's disease (vWd) was studied in five patients representing three generations of a single family. Bleeding times in the upper normal range in conjunction with low-normal platelet counts, normal factor VIII coagulant activity and VIII-related antigen, decreased VIII-ristocetin cofactor activity, selective decrease of the higher molecule weight factor VIII/von Willebrand factor (VIII/vWF) multimers, and increased ristocetin- induced platelet agglutination at low ristocetin concentrations were characteristic. Binding of patient VIII/vWF to washed normal platelets was within normal limits, whereas binding of normal VIII/vWF to patient platelets was significantly increased (p less than 0.001 at 0.6 mg/ml ristocetin). This disorder accordingly appears to involve an intrinsic platelet abnormality affecting platelet-VIII/vWF interactions. It is proposed that the concept of vWD be broadened to include patients with this abnormality, which may appropriately be called “Platelet-type von Willebrand's disease.” |
| Related Links | https://ashpublications.org/blood/article-pdf/60/3/790/211769/790.pdf |
| Ending Page | 794 |
| Page Count | 5 |
| Starting Page | 790 |
| DOI | 10.1182/blood.v60.3.790.bloodjournal603790 |
| Journal | Blood |
| Issue Number | 3 |
| Volume Number | 60 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 1982-09-01 |
| Access Restriction | Open |
| Subject Keyword | Hematology Platelet Bleeding Antigen Abnormality Von Vwd Ristocetin Viii/vwf Willebrand's Journal: Blood (Vol- 65, Issue- 3) |
| Content Type | Text |
| Resource Type | Article |
