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DOI: 10.1055/s-0038-1660152
Multicenter Comparison of von Willebrand Factor Multimer Sizing Techniques
Report of the Factor VIII and von Willebrand Factor SubcommitteePublication History
Received 21 August 1985
Accepted 21 August 1985
Publication Date:
19 July 2018 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00035024/198504/lookinside/thumbnails/10-1055-s-0038-1660152-1.jpg)
Summary
A multicenter study of various types of von Willebrand’s disease (vWD) was conducted in order to compare the different electrophoretic techniques used to evaluate von Willebrand factor multimers in plasma. Seven laboratories participated in the blind study of eight plasma samples from two healthy subjects and six vWD types and subtypes (la, lb, IIA, IIB, IIC and IID). From the results of the multimeric analysis of these samples, it appears that the differential diagnosis of vWD types and subtypes should be first approached by using a low-resolution electrophoretic technique, where each vWF multimer appears as a single band. Low-resolution techniques differentiate type I from type II, subtype la from lb and also subtype IIA from other type II subtypes. When type II subtypes other than IIA are identified with these techniques, samples should be rerun using high resolution techniques that resolve each of the fastest migrating multimers in at least three subbands, and permit the differentiation of subtypes IIB, IIC and IID.
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References
- 1 Ruggeri ZM, Zimmerman TS. Variant von Willebrand’s disease. Characterization of two subtypes by analysis of multimeric composition of factor VUI/von Willebrand factor in plasma and in platelets. J Clin Invest 1980; 65: 1318-1325
- 2 Hoyer LW, Shainoff JR. Factor VIII related protein circulates in normal human plasma as high molecular weight multimers. Blood 1980; 55: 1056-1059
- 3 Ruggeri ZM, Zimmerman TS. The complex multimeric composition of factor VUI/von Willebrand factor. Blood 1981; 57: 1140-1143
- 4 Kinoshita S, Harrison J, Lazerson J, Abildgaard CF. A new variant of dominant type II von Willebrand’s disease with aberrant multimeric pattern of factor VUI-related antigen (type IID). Blood 1984; 63: 1369-1371
- 5 Ruggeri ZM, Nilsson IM, Lombardi R, Holmberg L, Zimmerman TS. Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand’s disease (Type IIC). J Clin Invest 1982; 70: 1124-1127
- 6 Hoyer LW, Rizza CR, Tuddenham EGD, Carta CA, Armitage H, Rotblat F. von Willebrand factor multimer patterns in von Willebrand’s disease. Br J Haematol 1983; 55: 493-507
- 7 Ruggeri ZM, Pareti FI, Mannucci PM, Ciavarella N, Zimmerman TS. Heightened interaction between platelets and factor VUI/von Willebrand factor in a new subtype of von Willebrand’s disease. N Engl J Med 1980; 302: 1047-1051
- 8 Zimmerman TS, Dent JA, Federici AB, Ruggeri ZM, Nilsson IM, Holmberg L, Abildgaard CA, Nannini LH. High resolution NaDodSo4 agaroseelectrophoresis identifies new molecular abnormalities in von Willebrand’s disease. Clin Res 1984; 32: 501