Bellucci S, Levy-Toledano S, Maclouf J, Rendu F, Tobelem G, Caen J P
Scand J Haematol. 1986 Feb;36(2):142-6. doi: 10.1111/j.1600-0609.1986.tb00818.x.
A patient is described who presented a thrombocytopenia with thrombocytopathy followed by the development of a leukaemia. The disorder was characterized by decreased aggregation in the presence of ADP, and a lack of aggregation in the presence of arachidonic acid, natural endoperoxide or collagen. In parallel, 14C-serotonin release was severely decreased or nil in response to these inducers. Thrombin induced a slightly decreased aggregation and a normal 14C-serotonin release. Thromboxane B2 (T X B2) synthesis was normal after stimulation by arachidonic acid, natural endoperoxide or thrombin showing a normal arachidonate metabolism. In addition, the mepacrine test showed no significant decrease of the number of dense bodies with an average of 4.6 per platelet (versus 5.4 +/- 0.8 sd in controls). Stimulation by ionophore A 23187 failed to induce aggregation, 14C-serotonin release, or T X B2 synthesis. Furthermore, in the presence of EDTA, A 23187 did not provoke activation as reflected by 14C-serotonin release or T X B2 synthesis. Thus, in this case of thrombocytopathy, the hypothesis of abnormal intracellular Ca++ fluxes responsible for the defective platelet release phenomenon, was suggested.
本文描述了一名患者，该患者最初表现为血小板减少伴血小板病，随后发展为白血病。该病症的特征为：在二磷酸腺苷（ADP）存在时聚集减少，在花生四烯酸、天然内过氧化物或胶原存在时缺乏聚集。同时，对这些诱导剂的反应中，14C-5-羟色胺释放严重减少或为零。凝血酶诱导的聚集略有减少，但14C-5-羟色胺释放正常。花生四烯酸、天然内过氧化物或凝血酶刺激后，血栓素B2（TXB2）合成正常，表明花生四烯酸代谢正常。此外，吖啶黄试验显示致密体数量无显著减少，平均每个血小板有4.6个致密体（对照组为5.4±0.8标准差）。离子载体A23187刺激未能诱导聚集、14C-5-羟色胺释放或TXB2合成。此外，在乙二胺四乙酸（EDTA）存在下，A23187未引发14C-5-羟色胺释放或TXB2合成所反映的激活。因此，在这种血小板病病例中，有人提出细胞内钙离子通量异常导致血小板释放现象缺陷的假说。
