Nishida Mutsumi, Hirabayashi Shinsuke, Iwai Takahito, Sato Megumi, Kudo Yusuke, Omotehara Satomi, Horie Tatsunori, Sakano Ryosuke, Terashita Yukayo, Cho Yuko, Manabe Atsushi, Teshima Takanori
Management Strategy Department, Hokkaido University Hospital, N14 W5, Kita-ku, Sapporo, 060-8648, Japan.
Department of Pediatrics, Hokkaido University Hospital, Sapporo, Japan.
Int J Hematol. 2025 Apr 26. doi: 10.1007/s12185-025-03995-1.
Sinusoidal obstruction syndrome (SOS)/veno-occlusive disease (VOD) is a serious complication following hematopoietic stem cell transplantation. It is more common in children, with an incidence of approximately 20% compared to 10% in adult patients. While the Hokkaido ultrasonography (US)-based scoring system (HokUS-10 and HokUS-6) has proven effective in diagnosing SOS/VOD in adults, its utility in children remains uncertain. This study evaluated the utility of the HokUS-10/6 scoring systems in diagnosing pediatric SOS/VOD (pSOS/VOD) in a cohort of 99 patients, including 13 diagnosed with pSOS/VOD. Hyperbilirubinemia (> 2 mg/dL) was observed in only three patients (23%), whereas transfusion-refractory thrombocytopenia, hepatomegaly, and ascites were observed in all 13 patients. When the cutoff values for HokUS-10 and HokUS-6 scores were set at 4 and 2, the respective areas under the curve were 0.977 and 0.957. The sensitivity and specificity were 100% and 80% for HokUS-10, and 77% and 97% for HokUS-6, respectively. The simplified HokUS-6 may be a practical tool for diagnosing pSOS/VOD when use of the HokUS-10 scoring system is not feasible. HokUS-10/6 is important for early detection of pSOS/VOD during regular examinations and plays a valuable role in the diagnosis of clinical pSOS/VOD.
窦性阻塞综合征（SOS）/静脉闭塞性疾病（VOD）是造血干细胞移植后的一种严重并发症。它在儿童中更为常见，发病率约为20%，而成人患者的发病率为10%。虽然基于北海道超声（US）的评分系统（HokUS - 10和HokUS - 6）已被证明在诊断成人SOS/VOD方面有效，但其在儿童中的实用性仍不确定。本研究评估了HokUS - 10/6评分系统在99例患者队列中诊断儿童SOS/VOD（pSOS/VOD）的实用性，其中13例被诊断为pSOS/VOD。仅3例患者（23%）出现高胆红素血症（>2mg/dL），而所有13例患者均出现输血难治性血小板减少、肝肿大和腹水。当将HokUS - 10和HokUS - 6评分的临界值分别设定为4和2时，曲线下面积分别为0.977和0.957。HokUS - 10的敏感性和特异性分别为100%和80%，HokUS - 6的敏感性和特异性分别为77%和97%。当使用HokUS - 10评分系统不可行时，简化的HokUS - 6可能是诊断pSOS/VOD的实用工具。HokUS - 10/6对于在定期检查期间早期发现pSOS/VOD很重要，并且在临床pSOS/VOD的诊断中发挥着重要作用。
