Nieuwenhuis H K, Akkerman J W, Sixma J J
Blood. 1987 Sep;70(3):620-3.
One hundred six patients with storage pool deficiency (SPD) were studied with respect to platelet count, bleeding time, total platelet ATP and ADP, platelet serotonin, and in vitro aggregation. The diagnosis of SPD was made on basis of a prolonged bleeding time, a decreased total platelet ADP, and a diminished level of serotonin. Fifty-one patients from 34 unrelated families had congenital SPD, and 55 patients had acquired SPD. Congenital SPD was a common disorder in patients with a lifelong bleeding tendency and a prolonged bleeding time. The frequency in this group of patients was 18%, about one-half the frequency of von Willebrand's disease (vWd). Twenty-three percent of all patients had normal aggregation responses to ADP, epinephrine, and collagen; 33% had aggregation tracings typical for a secretion defect; and 44% had miscellaneous aggregation abnormalities. These findings indicate that SPD is common, heterogeneous, and not necessarily associated with in vitro aggregation abnormalities.
对106例储存池缺陷（SPD）患者进行了血小板计数、出血时间、血小板总ATP和ADP、血小板5-羟色胺及体外聚集方面的研究。SPD的诊断基于出血时间延长、血小板总ADP降低以及5-羟色胺水平降低。来自34个无亲缘关系家庭的51例患者患有先天性SPD，55例患者患有获得性SPD。先天性SPD是具有终生出血倾向和出血时间延长患者中的常见疾病。该组患者中的发生率为18%，约为血管性血友病（vWd）发生率的一半。所有患者中有23%对ADP、肾上腺素和胶原的聚集反应正常；33%具有分泌缺陷典型的聚集图谱；44%具有其他聚集异常。这些发现表明SPD很常见、具有异质性，且不一定与体外聚集异常相关。
