Holmsen H, Walsh P N, Koike K, Murphy S, Holme S, Johnson M M, Dangelmaier C A, Egan J J, Benzel J E, Tuszynski G P
Thrombosis Research Center, Temple University School of Medicine, Philadelphia, PA 19140.
Br J Haematol. 1987 Nov;67(3):335-44.
Aggregation responses to low concentrations of ADP, epinephrine, collagen and cationophore A23187 in platelets from two family members with marked bleeding tendencies were virtually absent, whereas shape change with ADP was normal. The contribution to factor X activation by collagen-treated platelets was markedly decreased. Glycoproteins IIb and III were also significantly reduced. The patients' platelets had normal stores of secretable constituents, but secretion of adenine nucleotides and acid hydrolases in response to low concentrations of thrombin and A23187 was drastically reduced compared to normal platelets; secretion of platelet factor 4 was normal. Agonist concentrations that normally produce maximal responses induced only partial aggregation and secretion in the patients' platelets. After prelabelling with [3H]arachidonate thrombin caused less changes in the [3H]phosphatidylinositol, [3H]phosphatidylcholine and free [3H]arachidonate in platelets from the patients than in platelets from normals. We conclude that the patients' platelets have an impairment in part of the signal processing mechanism that is common for all agonists and responses. This platelet abnormality, which has a superficial resemblance to thrombasthenia, represents a hitherto undescribed qualitative platelet disorder.
来自两名有明显出血倾向的家庭成员的血小板，对低浓度的二磷酸腺苷（ADP）、肾上腺素、胶原蛋白和离子载体A23187的聚集反应几乎不存在，而对ADP的形态变化正常。胶原蛋白处理的血小板对因子X激活的贡献明显降低。糖蛋白IIb和III也显著减少。患者血小板中可分泌成分的储存正常，但与正常血小板相比，对低浓度凝血酶和A23187的反应中腺嘌呤核苷酸和酸性水解酶的分泌大幅减少；血小板因子4的分泌正常。通常产生最大反应的激动剂浓度在患者血小板中仅诱导部分聚集和分泌。用[3H]花生四烯酸盐预标记后，凝血酶引起的患者血小板中[3H]磷脂酰肌醇、[3H]磷脂酰胆碱和游离[3H]花生四烯酸盐的变化比正常血小板中的小。我们得出结论，患者血小板的部分信号处理机制存在缺陷，这一缺陷对所有激动剂和反应来说是共有的。这种血小板异常与血小板无力症表面相似，代表了一种迄今未被描述的血小板质量性疾病。
