臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
症例
Type II Thrombastheniaの2症例
新名主 宏一丸山 芳一丸山 征郎納 光弘井形 昭弘
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1986 年 27 巻 4 号 p. 576-582

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Two cases of type II thrombasthenia (TA) were presented and investigated with special reference to the platelet-membrane glycoprotein abnormalities.
They were a 15-yr-old girl and a 33-yr-old woman suffering from bleeding tendeucy such as easy bruising, epistaxis and menorrhagia from infancy. They had quite similar platelet function abnormalities characterized by a markedly prolonged bleeding time without thrombocytopenia, and absent aggregation responses to ADP and other physiologic stimulating agents except for ristocetin or bovine factor VIII. Clot retraction and the interaction between platelets and polymerizing fibrin were normal. Tests of coaqulation/fibrinolysis were all normal. These results indicated that they were type II TA.
Analysis of platelet-membrane glycoproteins by one- or two-dimensional SDS-polyacrylamide gel electrophoresis revealed the corresponding abnormalities with type II TA, that is, not defective but decreased amounts of glycoprotein IIb and III (IIIa).

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© 1986 日本臨床血液学会
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