Lee Anselm Chi-Wai
Children's Haematology and Cancer Centre, Mount Elizabeth Hospital, Mount Elizabeth, Singapore.
J Pediatr Hematol Oncol. 2019 Jan;41(1):47-50. doi: 10.1097/MPH.0000000000001334.
An acquired, transient bleeding disorder that predominantly affects children in Southeast Asia has been reported for the last 4 decades. The condition has been named idiopathic purpura with gray platelets (IPGP) or acquired platelet dysfunction with eosinophilia. In a retrospective review from a private pediatric clinic over an 8-year period, 10 consecutive children were diagnosed as IPGP with a mean age of 8.4 (3.7 to 16.2) years. Eosinophilia (>0.5×10/L) was absent in 1, while gray platelets were consistently found in all cases with a mean proportion of 64.5% (40% to 80%). Platelet aggregation tests were performed in 9 patients with abnormal responses consistent with platelet storage pool defect. All children recovered completely and spontaneously from 1 to 4 months after diagnosis without specific therapy. In an otherwise well child who presents abruptly with easy bruising and a platelet count >100×10/L, IPGP can be readily recognized as an acquired form of gray platelet syndrome. Eosinophilia is common but not mandatory for diagnosis.
在过去40年里，有报道称一种主要影响东南亚儿童的后天性短暂出血性疾病。这种病症被命名为灰色血小板性特发性紫癜（IPGP）或嗜酸性粒细胞增多伴获得性血小板功能障碍。在一家私立儿科诊所进行的一项为期8年的回顾性研究中，连续10名儿童被诊断为IPGP，平均年龄为8.4（3.7至16.2）岁。其中1名儿童无嗜酸性粒细胞增多（>0.5×10⁹/L），而所有病例均持续发现灰色血小板，平均比例为64.5%（40%至80%）。对9例患者进行了血小板聚集试验，结果显示反应异常，符合血小板储存池缺陷。所有儿童在诊断后1至4个月内未经特殊治疗完全自发康复。对于一名原本健康但突然出现易瘀伤且血小板计数>100×10⁹/L的儿童，IPGP可被轻易识别为获得性灰色血小板综合征。嗜酸性粒细胞增多很常见，但不是诊断的必要条件。
