Phadke K, Dean S, Pitney W R
Am J Hematol. 1981;10(1):57-64. doi: 10.1002/ajh.2830100109.
The most common abnormality of the platelets in 43 patients with a variety of myeloproliferative syndromes was impaired or absent aggregation when stimulated with collagen and adrenaline. Eight unselected cases studied in more detail showed a normal prostaglandin synthesis pathway as evidenced by normal aggregation with arachidonic acid and the production of normal amounts of malonyldialdehyde. Mixing experiments with aspirin-tested platelets showed correction of the abnormal adrenaline and collagen responses and confirmed that the nature of the defect was different from that induced by aspirin. Stimulation of "myeloproliferative" platelets with thrombin after blocking the prostaglandin pathway with aspirin resulted in reduced aggregation, indicating either a deficiency of the storage pool of adenine nucleotides in the platelets or an abnormality of a membrane receptor for thrombin.
43例患有各种骨髓增殖性综合征患者中，血小板最常见的异常情况是在受到胶原蛋白和肾上腺素刺激时，聚集功能受损或缺失。对8例未经挑选的病例进行更详细研究发现，前列腺素合成途径正常，这可通过花生四烯酸诱导的正常聚集以及正常量丙二醛的产生得以证明。对用阿司匹林检测过的血小板进行的混合实验显示，异常的肾上腺素和胶原蛋白反应得到纠正，并证实该缺陷的性质与阿司匹林诱导的不同。在用阿司匹林阻断前列腺素途径后，用凝血酶刺激“骨髓增殖性”血小板，导致聚集减少，这表明要么血小板中腺嘌呤核苷酸储存池存在缺陷，要么凝血酶膜受体存在异常。
