Heyns A du P, Badenhorst P N, Wessels P, Pieters H, Lötter M G
Br J Haematol. 1985 Jun;60(2):323-30. doi: 10.1111/j.1365-2141.1985.tb07418.x.
Six patients with giant platelet syndrome were examined: four with Bernard-Soulier syndrome (two were asplenic); one with hereditary thrombopathic thrombocytopenia; and one with May-Hegglin anomaly. Autologous platelets were labelled with In-111-oxine and in vivo redistribution and sites of sequestration measured with quantitative imaging. In Bernard-Soulier syndrome platelet survival was normal or moderately shortened; platelet turnover was decreased only in the two patients with thrombocytopenia. In the patients with thrombopathia or May-Hegglin anomaly, platelet survival and turnover was moderately decreased. In those patients with normal-sized spleens, the mean splenic platelet pool consisted of 35.5% of the platelet mass, i.e. normal. The intrasplenic transmit time of the megathrombocytes was prolonged. Splenic blood flow was within normal limits. There was a marked accumulation of platelets in the liver at equilibrium: 15.5-58.8% of whole body radioactivity (normal 9.6 +/- 1.2%). This finding is unexplained. The final sites of sequestration of platelets were mainly in the liver and spleen, similar to that seen in normal subjects. We conclude that there is no inverse relationship between cell size and splenic platelet transit time. Platelet size therefore does not determine the size of the splenic platelet pool. The size of the platelets also does not seem to affect the sites of sequestration at the end of their life span.
对6例巨大血小板综合征患者进行了检查：4例为伯纳德-索利尔综合征（其中2例无脾）；1例为遗传性血小板病性血小板减少症；1例为梅-赫格琳异常。用铟-111-奥克辛标记自体血小板，并通过定量成像测量体内再分布和滞留部位。在伯纳德-索利尔综合征中，血小板生存期正常或中度缩短；仅2例血小板减少患者的血小板周转率降低。在血小板病或梅-赫格琳异常患者中，血小板生存期和周转率中度降低。在脾脏大小正常的患者中，平均脾脏血小板池占血小板总量的35.5%，即正常。巨血小板在脾内的传输时间延长。脾血流量在正常范围内。平衡时肝脏中有明显的血小板积聚：占全身放射性的15.5 - 58.8%（正常为9.6±1.2%）。这一发现无法解释。血小板最终的滞留部位主要在肝脏和脾脏，与正常受试者相似。我们得出结论，细胞大小与脾脏血小板传输时间之间不存在反比关系。因此，血小板大小不能决定脾脏血小板池的大小。血小板大小似乎也不影响其寿命末期的滞留部位。
