Von Willebrand's disease has been thought to be hereditary bleeding disorder. Recently, about 14 cases of acquired von Willebrand's syndrome were reported in the world. Most of them are associated with immunological disorders such as SLE, monoclonal gammopathy, myeloma and CLL.
In this paper, a 61 year-old woman was described with laboratory data of von Willebrand's syndrome and gastrointestinal bleeding. She had no past and family history of abnormal bleeding.
Laboratory data showed low F. VIII coag, low F. VIII ag, but bleeding time was varied from normal to mildly prolonged value, and PL count and PL adhesiveness were both normal.
Ristocetin induced platelet aggregation was nil, and F. VIII vwf measured with the method of Weiss revealed low value. Later, she suddenly collapsed with massive hematemesis, and was cured by total gastrectomy in emergency with transfusion of AHF.
This is provably the first case of acquired von Willebrand's syndrome with gastrointestinal bleeding unassociated with basic disorder in Japan.
