Department of Pediatrics, Baylor College of Medicine, Houston, TX; Section of Hematology/Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX.
Haemophilia. 2014 Mar;20(2):249-54. doi: 10.1111/hae.12293. Epub 2013 Nov 20.
Platelet function defects (PFD) are reported to occur frequently in adult women with heavy menstrual bleeding (HMB). Few studies on adolescent HMB report varying incidence rates (2-44%) for PFD. We reviewed our institutional experience in detecting and managing PFD in adolescent HMB. Postmenarchial girls and adolescents with HMB seen at our institution undergo a comprehensive bleeding disorder work-up by paediatric haematology and paediatric gynaecology providers. Whole blood platelet aggregometry (WBPA) is performed as a second tier test after excluding thrombocytopaenia, coagulation factor deficiencies and Von Willebrand disease (VWD). We retrospectively reviewed the medical records of adolescents with HMB seen between June 2009 and November 2010, as approved by the Institutional Review Board. Patient demographics, clinical features, laboratory results, therapy details and patient outcome information were analysed. Overall, 114 postmenarchial girls and adolescents with HMB were evaluated; 68 patients (59%) had WBPA study performed. Nineteen patients (28%) had at least one aggregation or secretion defect; 12 (18%) had two or more such defects. Treatment included hormonal therapy (13/19; 68%), antifibrinolytic agents (8/19; 42%) and intra-nasal DDAVP (3/19; 16%). Thirteen patients (81%) had improved outcome (median follow-up--15.6 months; range of 1-66 months). In this study, PFD were identified in nearly one-third of girls with HMB, with the majority of these having two or more defects as identified by WBPA. Further prospective studies are needed to better define the prevalence and address appropriate management of HMB and other bleeding complications of PFD in adolescents.
血小板功能缺陷(PFD)在月经过多(HMB)的成年女性中经常发生。少数关于青少年 HMB 的研究报告 PFD 的发病率不同(2-44%)。我们回顾了我们在检测和管理青少年 HMB 中的 PFD 方面的机构经验。在我们的机构中,初潮后的女孩和 HMB 的青少年接受儿科血液学和儿科妇科医生的全面出血性疾病检查。全血血小板聚集(WBPA)是在排除血小板减少症、凝血因子缺乏症和血管性血友病(VWD)后作为二线检查进行的。我们回顾性分析了 2009 年 6 月至 2010 年 11 月间在我们机构就诊的 HMB 青少年患者的病历,该研究得到了机构审查委员会的批准。分析了患者的人口统计学、临床特征、实验室结果、治疗细节和患者结局信息。总体上,评估了 114 名 HMB 的初潮后女孩和青少年,其中 68 名患者(59%)进行了 WBPA 研究。19 名患者(28%)至少有一种聚集或分泌缺陷;12 名(18%)有两种或更多种此类缺陷。治疗包括激素治疗(19/19;68%)、抗纤维蛋白溶解剂(19/19;42%)和鼻内 DDAVP(19/19;16%)。13 名患者(81%)有改善的结局(中位随访时间为 15.6 个月;范围为 1-66 个月)。在这项研究中,近三分之一的 HMB 女孩存在 PFD,其中大多数通过 WBPA 发现有两种或更多种缺陷。需要进一步的前瞻性研究来更好地确定青少年 HMB 和 PFD 的其他出血并发症的患病率,并解决适当的管理问题。