Loading...
Please wait, while we are loading the content...
Similar Documents
Selective absence of large forms of factor VIII/von Willebrand factor in acquired von Willebrand's syndrome. Response to transfusion
| Content Provider | Scilit |
|---|---|
| Author | Meyer, D. Frommel, D. Larrieu, Mj Zimmerman, Ts |
| Copyright Year | 1979 |
| Description | A previously healthy elderly man with mucocutaneous bleeding was found to have a benign monoclonal IgG gammapathy associated with criteria for severe von Willebrand disease (Factor VIII procoagulant activity, Factor-VIII-related antigen, and ristocetin cofactor activity, less than 10% of normal). Associated qualitative abnormalities of factor VIII/von Willebrand factor were demonstrated by radiocrossed immunoelectrophoresis and immunoradiometric assay. The late clinical onset and negative family history are in favor of an acquired form of vWD. The monoclonal gammapathy and abnormalities of factor VIII/von Willebrand factor have been stable over a 10-yr period. No inhibitor to Factor VIII procoagulant activity, ristocetin cofactor activity, or Factor-VIII-related antigen could be demonstrated. Following transfusion of cryoprecipitate (with a normal cross immunoelectrophoretic pattern), there was a rapid removal of the large forms of Factor.-VIII-related antigen, paralleled by a decay of ristocetin cofactor activity. The transfusion study of this patient with acquired von Willebrand disease type II (variant of von Willebrand disease) serves to emphasize the relationship between polydispersity of Factor VIII/von Willebrand Factor and functional heterogeneity. |
| Related Links | https://ashpublications.org/blood/article-pdf/54/3/600/582492/600.pdf |
| Ending Page | 606 |
| Page Count | 7 |
| Starting Page | 600 |
| DOI | 10.1182/blood.v54.3.600.600 |
| Journal | Blood |
| Issue Number | 3 |
| Volume Number | 54 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 1979-09-01 |
| Access Restriction | Open |
| Subject Keyword | Hematology Von Willebrand Disease Antigen Transfusion Willebrand Factor Factor Viii/von Viii/von Willebrand Journal: Blood (Vol- 64, Issue- 3) |
| Content Type | Text |
| Resource Type | Article |
