Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey.
Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey.
Transfus Apher Sci. 2024 Jun;63(3):103909. doi: 10.1016/j.transci.2024.103909. Epub 2024 Mar 8.
Hepatic sinusoidal obstruction syndrome (SOS) is an illness with serious life effects that develops after hematopoietic stem cell transplantation (HSCT). We investigated the risk factors and clinical features of hepatic SOS in children following HSCT in 210 children who underwent allogeneic or autologous HSCT between 2009 and 2021 were analyzed in the context of SOS. The syndrome developed in 22 (10.4%) patients:frequently in neuroblastoma [24% (5/21)], hemophagocytic lymphohistiocytosis [57% (4/7)], and thalassemia major [22% (7/31)]. The median time from HSCT to diagnosis was 16 (6-38) days. Severe disease occurred in 8 (36%) patients, and mild/moderate in 14 (64%) and 4 patients died (18%). In univariate analyses, patient's age ≤ 2 years [odds ratio (OR)= 3.043, P = 0.028], pretransplant AST and ALT levels > 100 U/L (OR=3.576, P = 0.045), and chemotherapy/radiotherapy to abdomen before transplantation (OR = 3.162, P = 0.044) were determined as risk factors. In multivariate analysis, pre-transplant AST and ALT levels > 100 U/L (OR = 16.04, P = 0.010) and ferritin levels over 1000 mg/dl (OR=5.15, P = 0.047) were significant. The only independent risk factor on mortality was the age ≤ 2 years (P = 0.001). Although our study confirmed several risk factors for SOS, we failed to achieve some well-known risk factors. Precautions should be taken considering the factors affecting liver function before transplantation and the risk of SOS in infants receiving chemotherapy and radiotherapy before transplantation, such as neuroblastoma in which comparable results in respect to the chemotherapy only. The risk factors should be fully elucidated in multicenter studies to improve preventive and therapeutic strategies.
肝窦阻塞综合征(SOS)是一种造血干细胞移植(HSCT)后严重影响生命的疾病。我们研究了 2009 年至 2021 年期间接受异基因或自体 HSCT 的 210 例儿童中 SOS 相关的 HSCT 后儿童的 SOS 的危险因素和临床特征。22 例(10.4%)患者出现了该综合征:神经母细胞瘤(24%,5/21)、噬血细胞性淋巴组织细胞增多症(57%,4/7)和重型地中海贫血(22%,7/31)较为常见。从 HSCT 到诊断的中位时间为 16(6-38)天。8 例(36%)为重症疾病,14 例(64%)为轻度/中度,4 例死亡(18%)。在单因素分析中,患者年龄≤2 岁[比值比(OR)=3.043,P=0.028]、移植前 AST 和 ALT 水平>100 U/L(OR=3.576,P=0.045)和移植前腹部化疗/放疗(OR=3.162,P=0.044)被确定为危险因素。多因素分析中,移植前 AST 和 ALT 水平>100 U/L(OR=16.04,P=0.010)和铁蛋白水平>1000 mg/dl(OR=5.15,P=0.047)是显著的。死亡的唯一独立危险因素是年龄≤2 岁(P=0.001)。尽管我们的研究证实了 SOS 的几个危险因素,但我们未能确定一些众所周知的危险因素。在考虑移植前影响肝功能的因素和接受化疗和放疗的婴儿发生 SOS 的风险时,应采取预防措施,例如神经母细胞瘤,其中仅化疗的结果相似。应在多中心研究中充分阐明危险因素,以改善预防和治疗策略。