[Diagnostic strategies for detection of the von Willebrand syndrome].
The von Willebrand disease (vWD) is the most severe coagulopathy. Because of the complex biochemical structure of the von Willebrand factor (vWF), a great number of types and subtypes of the vWD were found. A screening of vWD can only be done by examining the bleeding time, the ristocetin cofactor activity (risto) and by an immunological determination of the vWF concentration. Examinations of 200 patients with a bleeding tendency showed that the ratio vWF/risto < 0.7 indicates a high probability for an abnormal multimeric structure of vWF. The exact determination of the vWD subtype then has to be done by a SDS-agarose gel electrophoresis. In 16.8% of our patients we found a decreased vWF concentration in the platelets. These patients showed normal plasmatic coagulation factors, but a bleeding tendency and a prolonged bleeding time. For diagnosis of vWD the bleeding time, immunological determination of the vWF and the risto should be done first. If a ratio vWF/risto < 0.7 or a prolonged bleeding time with a bleeding tendency is found, the separation of the vWF multimers into plasma and platelets and the determination of the vWF concentration in platelets should be carried out for an exact diagnosis of vWD.
Eller
T
T
Abteilung für klinische Chemie und Zentrallabor, Universitätsklinik Marburg, BRD.
Mayer
J
J
Bomhard
M
M
Keller
F
F
D001780:Blood Coagulation Tests; D001782:Blood Donors; D004797:Enzyme-Linked Immunosorbent Assay; D006801:Humans; D010976:Platelet Count; D012016:Reference Values; D014842:von Willebrand Diseases / Q000097:blood / Q000175:diagnosis*; D014841:von Willebrand Factor / Q000032:analysis
D014841:von Willebrand Factor
Diagnostische Strategien zur Erkennung des von-Willebrand-Syndroms.