Institute for Inflammation Research, Department of Rheumatology and Spine Disease, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Department of Pediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Bone Marrow Transplant. 2019 Sep;54(9):1406-1418. doi: 10.1038/s41409-018-0426-8. Epub 2019 Jan 25.
Sinusoidal obstruction syndrome (SOS) is a potentially life-threatening complication of allogeneic hematopoietic stem cell transplantation (HSCT). We assessed the proposed pediatric EBMT criteria along with the Baltimore and modified Seattle criteria in a population-based cohort. Eighty-seven children (1.1-17.3 years) undergoing myeloablative HSCT from 2010 to 2017 were consecutively included at the Danish National Transplantation Center. In total, 39 (44.8%) patients fulfilled the EBMT criteria and 30 patients (35%) fulfilled the criteria for severe or very severe SOS. Nine (10.3%) patients fulfilled the modified Seattle criteria while none met the Baltimore criteria. Patients fulfilling the EBMT criteria for SOS had longer primary admission (31 days (23-183) vs. 27 days (17-61), p = 0.001), were treated more intensively with diuretics within the first 3 months (29 days (0-90) vs. 3.5 days (0-90), p < 0.0001), and had a longer time to stable platelet counts >50 × 10/L (32 days (16-183) vs. 23 days (14-101), p < 0.0001). Two patients, fulfilling neither Baltimore nor Seattle criteria, but selectively fulfilling EBMT criteria, died of treatment-related acute inflammatory complications within 1 year post-HSCT. In conclusion, application of the pediatric EBMT diagnostic and severity criteria may be helpful in identifying patients at increased risk of severe treatment-related complications and mortality, although with a risk of over-diagnosing SOS.
窦状隙阻塞综合征（SOS）是异基因造血干细胞移植（HSCT）的一种潜在危及生命的并发症。我们评估了基于人群队列的小儿 EBMT 标准以及巴尔的摩和改良西雅图标准。2010 年至 2017 年，丹麦国家移植中心连续纳入 87 名（1.1-17.3 岁）接受清髓性 HSCT 的儿童。共有 39 名（44.8%）患者符合 EBMT 标准，30 名患者（35%）符合严重或极严重 SOS 的标准。9 名（10.3%）患者符合改良西雅图标准，而无 1 名患者符合巴尔的摩标准。符合 EBMT SOS 标准的患者初次住院时间更长（31 天（23-183）vs. 27 天（17-61），p=0.001），在最初 3 个月内使用利尿剂治疗更密集（29 天（0-90）vs. 3.5 天（0-90），p<0.0001），血小板计数稳定>50×10/L 的时间更长（32 天（16-183）vs. 23 天（14-101），p<0.0001）。2 名患者既不符合巴尔的摩标准也不符合西雅图标准，但选择性地符合 EBMT 标准，在 HSCT 后 1 年内死于治疗相关的急性炎症并发症。总之，应用小儿 EBMT 诊断和严重程度标准可能有助于识别发生严重治疗相关并发症和死亡率风险增加的患者，尽管存在 SOS 过度诊断的风险。
