Platelet function in a patient with thrombasthenia.
Platelet function in a patient with thrombasthenia.
复制标题
血小板无力症患者的血小板功能。
作者：
M. Zucker;M. Zucker;J. Pert;M. Hilgartner;M. Hilgartner
先天性血小板无力症患者的血小板不聚集ADP，凝血酶，结缔组织颗粒，聚凝胺，或磷脂酶C，并没有坚持无论是在玻璃载玻片上测量或保留在玻璃珠柱的玻璃。血凝块回缩明显减少。提高镁水平部分纠正了血凝块收缩，但没有恢复ADP诱导的结块。血小板促进凝血酶原消耗的能力较低。冻融血小板上清液中纤维蛋白原浓度较低，但表面纤维蛋白原似乎正常。血小板失活症的血小板在以下方面是正常的：ATP和甘油醛-3-磷酸脱氢酶的浓度;与结缔组织纤维的粘附;抗血小板血清的聚集;微电泳迁移率;等电点; 37 ℃时血小板的盘形;血小板随ADP或寒冷改变形状的能力;凝血酶引起的ATP浓度降低和颗粒金胺染色; 5-羟色胺、ADP和其他吸收260 mµ的物质的释放。凝血酶或结缔组织颗粒;血液凝固过程中酸性磷酸酶的释放;血小板因子5活性。结果表明，血小板对凝血酶和结缔组织颗粒的反应是正常的，只是释放的ADP不能引起聚集。对ADP反应的第一阶段，即从盘状到棘状球的转化是正常的。在分子水平上仍有待确定的是凝块收缩和ADP诱导的聚集失败的原因以及这些缺陷与血小板提取物的低纤维蛋白原浓度的关系。
The platelets of a patient with congenital thrombasthenia were not aggregated by ADP, thrombin, connective tissue particles, Polybrene, or phospholipase C, and did not adhere to glass as measured either on a glass slide or by retention in a glass-bead column. Clot retraction was markedly diminished. Raising the magnesium level partially corrected clot retraction but did not restore ADP-induced clumping. The platelets were less able to promote prothrombin consumption. Fibrinogen concentration in the supernatant of frozen and thawed platelets was low, but surface fibrinogen appeared to be normal. The thrombasthenic platelets were normal in the following respects: concentration of ATP and glyceraldehyde-3-phosphate-dehydrogenase; adhesion to connective tissue fibers; aggregation by antiplatelet serum; microelectrophoretic mobility; isoelectric point; disc shape of platelets at 37 C.; ability of platelets to change shape with ADP or cold; decrease in ATP concentration and auramine staining of granules by thrombin; release of serotonin, ADP, and other materials absorbing at 260 mµ. by thrombin or connective tissue particles; liberation of acid phosphatase during blood clotting; and platelet Factor 5 activity. It is concluded that responses of thrombasthenic platelets to thrombin and connective tissue particles are normal except that the liberated ADP fails to cause aggregation. The first stage of the reaction to ADP, transformation from disc to spiny sphere, is normal. Still to be determined at the molecular level is the cause(s) of failure of clot retraction and ADP-induced aggregation and the relationship of these defects to the low fibrinogen concentration of platelet extracts.