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A new variant of dominant type II von Willebrand's disease with aberrant multimeric pattern of factor VIII-related antigen (Type IID). Blood (1984)
| Content Provider | CiteSeerX |
|---|---|
| Author | Abildgaard, Charles F. Harrison, Janet Lazerson, Jack Kinoshita, Seiji |
| Abstract | A new type II variant form of von Willebrand’s disease has been recognized in a mother and daughter who have bleeding manifestations typical of von Willebrand’s dis-ease. Laboratory findings include consistently prolonged bleeding times. with normal levels of factor VIII procoagu-lant and antigen. but decreased ristocetin cofactor activity. Electrophoresis in SDS 1.5 % agarose gel and reaction with 125l-labeled anti-factor VIII-related antigen rabbit lgG. fol-lowed by autoradiography. revealed that both plasma and F ACTOR VIII-related antigen (FVIIIR:Ag) ex-ists in normal plasma as a series of multimers with molecular weights from 0.9 x 106 to 20 x 106 daltons, as demonstrated by use of sodium dodecyl sulfate (SDS) agarose 1,2 By use of this technique, patients with von Willebrand’s disease (vWD) have been classified into several types. In the “classic ” form, or type I vWD, the multimer pattern is identical to normal plasma, but the total amount of antigen is decreased, ranging from complete absence (homozygote or double heterozygote) to a mild decrease. Type II vWD is characterized as lack-ing the large multimers, with two subtypes based on differences in reactivity to ristocetin (hA normal response, IIB increased response), and one subtype, IIC, characterized by an abnormally migrating mul-timeric pattern in 2.5 % agarose gel.3’4 Using similar methods, we have identified a new type II vWD, characterized by a multimeric pattern in 3.0 % agarose gel that has a slightly slower mobility of the major bands and three abnormally migrating sub-bands. The pattern of inheritance of the abnormality appears to be dominant, in that the prospositus and her daughter have identical findings. |
| File Format | |
| Publisher Date | 1984-01-01 |
| Access Restriction | Open |
| Content Type | Text |
