Platelet-type von Willebrand's disease: characterization of a new bleeding disorder
نویسندگان
چکیده
منابع مشابه
Platelet-type von Willebrand's disease: characterization of a new bleeding disorder.
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 c...
متن کاملFurther characterization of platelet-type von Willebrand's disease in Japan.
We studied four patients who showed aggregation of platelets in platelet-rich plasma at lower concentrations of ristocetin than those required for normal platelet-rich plasma and who demonstrated an increased capacity of the platelets to bind normal von Willebrand factor. The four patients were from two Japanese families. Platelets from one family aggregated spontaneously in vitro, and platelet...
متن کاملPlatelet von Willebrand factor: an important determinant of the bleeding time in type I von Willebrand's disease.
We studied 17 patients with moderate to mild type I von Willebrand's disease (vWd) and correlated the bleeding time with the plasma von Willebrand factor antigen (vWf Ag), the plasma vWf activity (ristocetin cofactor), the platelet vWf Ag, and the platelet vWf activity. We found an excellent correlation between the bleeding time and the platelet vWf activity and, to a lesser extent, between the...
متن کاملDiagnosis of platelet-type von Willebrand disease by flow cytometry.
Platelet-type von Willebrand disease (PT-VWD) is a rare autosomal dominant bleeding disorder which is due to a mutation in the gene encoding for platelet glycoprotein Ibalpha (GPIbalpha) resulting in enhanced affinity for von Willebrand factor (VWF). PT-VWD is often mistakenly diagnosed as type 2B VWD for the similarities between these two conditions. We characterized a new case of PT-VWD and e...
متن کاملUnique interactions of asialo von Willebrand factor with platelets in platelet-type von Willebrand disease.
The present studies demonstrate that platelets from patients with platelet-type von Willebrand disease show specific and saturable binding of asialo von Willebrand factor (AS-vWF) under conditions where such binding is not observed with normal platelets. Although specific binding of 125I-AS-vWF to formalin-fixed normal platelets could not be demonstrated, specific binding to fixed patient plate...
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ژورنال
عنوان ژورنال: Blood
سال: 1982
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v60.3.790.790