Department of Pediatrics, Jena University Hospital, Am Klinikum 1, 07747, Jena, Germany.
Comprehensive Cancer Center Central Germany (CCCG), Jena, Germany.
J Cancer Res Clin Oncol. 2024 Jan 11;150(1):9. doi: 10.1007/s00432-023-05561-w.
PURPOSE: Hepatic sinusoidal obstruction syndrome (SOS) is a serious complication following hematopoietic stem cell transplantation (HSCT) in which early diagnosis improves patient outcome. The aim of our study was to detect laboratory parameters following HSCT that can predict the occurrence of SOS. METHODS: This retrospective study included 182 children, adolescents, and young adults who underwent allogeneic or autologous HSCT for the first time (median age 7.2 years). The diagnosis of SOS was based on the pediatric criteria of European Society for Blood and Marrow Transplantation (EBMT). We investigated 15 laboratory parameters after HSCT before the onset of SOS. RESULTS: The overall incidence of SOS was 14.8%. SOS developed in 24 of 126 allogeneic (19.1%) and in 3 of 56 autologous (5.4%) HSCT patients at a median time of 13 days after HSCT. We observed a low SOS mortality rate of 11.1% within 100 days after HSCT. International normalized ratio (INR) ≥ 1.3, activated partial thromboplastin time (aPTT) ≥ 40 s, reptilase time ≥ 18.3 s, factor VIII ≤ 80%, antithrombin III ≤ 75%, protein C ≤ 48%, D-dimer ≥ 315 µg/L, bilirubin ≥ 9 µmol/L, and ferritin ≥ 3100 µg/L showed significant associations with the onset of SOS in the univariate analyses. In the multivariate analysis, INR ≥ 1.3 [odds ratio (OR) = 8.104, p = 0.006], aPTT ≥ 40 s (OR = 10.174, p = 0.001), protein C ≤ 48% (OR = 5.215, p = 0.014), and ferritin ≥ 3100 µg/L (OR = 7.472, p = 0.004) could be confirmed as independent risk factors after HSCT before SOS. If three of the four significant cut-off values were present, the probability of developing SOS was more than 70%. The probability of SOS was 96%, if all four laboratory parameters were changed according to the cut-off values. The values of factor XIII, von Willebrand factor (vWF), von Willebrand factor activity (vWF activity), protein S, fibrinogen, and alanine aminotransferase (ALT) were not relevant for the occurrence of SOS. CONCLUSION: In summary, the laboratory parameters INR, aPTT, protein C, and ferritin were very useful to predict the occurrence of SOS. In addition, this is the first report on a significant association between SOS and high values of INR and aPTT after HSCT before SOS.
目的：肝窦阻塞综合征（SOS）是造血干细胞移植（HSCT）后严重的并发症，早期诊断可改善患者预后。本研究旨在检测 HSCT 后可预测 SOS 发生的实验室参数。
方法：本回顾性研究纳入了 182 名首次接受异基因或自体 HSCT 的儿童、青少年和年轻成人（中位年龄 7.2 岁）。SOS 的诊断基于欧洲血液和骨髓移植学会（EBMT）的儿科标准。我们在 SOS 发病前 HSCT 后检测了 15 项实验室参数。
结果：SOS 的总发生率为 14.8%。在 126 例异基因（19.1%）和 56 例自体（5.4%）HSCT 患者中，SOS 分别在 HSCT 后中位时间 13 天出现。我们观察到 HSCT 后 100 天内 SOS 死亡率为 11.1%。国际标准化比值（INR）≥1.3、活化部分凝血活酶时间（aPTT）≥40 s、复钙时间≥18.3 s、因子 VIII≤80%、抗凝血酶 III≤75%、蛋白 C≤48%、D-二聚体≥315 μg/L、胆红素≥9 μmol/L 和铁蛋白≥3100 μg/L 在单因素分析中与 SOS 发病显著相关。多因素分析显示，INR≥1.3[比值比（OR）=8.104，p=0.006]、aPTT≥40 s（OR=10.174，p=0.001）、蛋白 C≤48%（OR=5.215，p=0.014）和铁蛋白≥3100 μg/L（OR=7.472，p=0.004）是 HSCT 后 SOS 发生的独立危险因素。如果在 SOS 之前存在四个显著截断值中的三个，那么发生 SOS 的概率超过 70%。如果根据截断值改变四项实验室参数，则发生 SOS 的概率为 96%。因子 XIII、血管性血友病因子（vWF）、vWF 活性（vWF 活性）、蛋白 S、纤维蛋白原和丙氨酸氨基转移酶（ALT）的数值与 SOS 的发生无关。
结论：总之，INR、aPTT、蛋白 C 和铁蛋白等实验室参数对预测 SOS 的发生非常有用。此外，这是首例报告 HSCT 前 SOS 发生时 INR 和 aPTT 升高与 SOS 显著相关。
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