Mori K, Suzuki S, Sugai K, Hisa S, Sakai H, Kobayashi Y
Tohoku J Exp Med. 1981 Apr;133(4):479-80. doi: 10.1620/tjem.133.479.
Bernard-Soulier Syndrome is characterized morphologically by many giant platelets in the peripheral blood. However, the mechanism of large platelet production has not yet been clarified. Our electron microscopic examinations revealed that some platelets have two or three definite circumferential bundles of microtubules, whereas no pathological findings were obtained in the liberation of platelets from megakaryocytes by light microscopy. From these observations, we consider that the giant platelets of Bernard-Soulier Syndrome are formed by fusion of two or three platelets while they are circulating in the peripheral blood stream.
伯纳德-索利尔综合征在外周血中表现为形态学上有许多巨大血小板。然而，大血小板产生的机制尚未阐明。我们的电子显微镜检查显示，一些血小板有两束或三束明确的周缘微管束，而通过光学显微镜观察巨核细胞释放血小板时未发现病理改变。基于这些观察结果，我们认为伯纳德-索利尔综合征的巨大血小板是由两三个血小板在外周血流中循环时融合形成的。
