45.病人有出血問題,其血小板流式細胞儀檢查結果如下圖,則病人最可能之診斷為何?

【Jin】評論
Bernard Soulier syndrome 是因為Ib表現量減少,而左圖是因為Ib有缺陷,所以才跑到陰性去了,故選A
【etrnya】評論
Bernard–Soulier syndrome (BSS), also called hemorrhagiparous thrombocytic dystrophy,[2] is a rare autosomal recessivecoagulopathy (bleeding disorder) that causes a deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor.[3]