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本文收集106例贮存池缺陷(SPD)患者,先天性SPD 51例,获得性SPD 55例。诊断标准:1.出血时间延长;2.血小板总ADP减低;3.血小板血清素水平减低。正常或轻度异常的血小板聚集曲线在少数先天性SPD有过报道,但获得性SPD未见报道。1980年,作者发现1例出血时间延长,聚集试验正常的SPD。之后,对不能解释的出血时间延长患者均进行研究。13例先天性SPD和10例获得性SPD对ADP、肾上腺素、胶原聚集反应正常(A型),17例先天性SPD和17例获得性SPD对小剂量和大剂量ADP及肾上腺素缺乏第二相聚集波,对小剂量胶原聚集反应轻微,对大剂量胶原聚集反应正常或减低,即典型分泌缺陷
This article collected 106 patients with storage pool defects (SPD), 51 cases of congenital SPD, acquired 55 cases of SPD. Diagnostic criteria: 1. Bleeding prolonged; 2. Total platelet decreased ADP; 3. Serum platelet serotonin levels decreased. Normal or mild abnormal platelet aggregation curves have been reported in a few congenital SPD, but acquired SPD has not been reported. In 1980, the authors found that one patient had prolonged bleeding and gathered normal SPD. Later, unexplained patients with prolonged bleeding were studied. Thirteen cases of congenital SPD and 10 cases of acquired SPD had normal ADP, adrenaline and collagen accumulation (type A), 17 cases of congenital SPD and 17 cases of acquired SPD. The second and third doses of ADP and epinephrine were the second Aggregation waves, the small dose of collagen aggregation reaction was mild, normal or reduced high-dose collagen aggregation, the typical secretion defects