Department of Hematology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Bone Marrow Transplant. 2019 Aug;54(8):1361-1368. doi: 10.1038/s41409-019-0492-6. Epub 2019 Feb 26.
Traditional severity criteria of sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) were determined retrospectively but found inappropriate for therapeutic decisions. Data of 203 patients with SOS/VOD were collected according to the modified Seattle diagnostic criteria and were analyzed for validation of the revised severity criteria recently proposed from European Society for Blood and Marrow Transplantation (EBMT). According to the traditional severity criteria, none of the patients were mild grade, while 63.1% were moderate and 36.9% were severe grade. However, according to the revised EBMT criteria, the majority of patients (63.1%) were very severe, 18.2% were severe, 12.8% were moderate, and 5.9% were mild grade. The 100-day overall survival (OS) of mild, moderate, severe and very severe groups was 83.3, 84.3, 94.6, and 58.6%, respectively. Very severe SOS/VOD showed a significantly lower OS than the others (58.6 vs. 89.3%, p < 0.0001). The 100-day transplantation-related mortality was 25.2% in the entire cohort; 8.3% in mild, 8.0% in moderate, 2.7% in severe, and 36.7% in very severe SOS/VOD (p < 0.0001). The very severe grade newly classified by the revised EBMT criteria accounted for the majority of SOS/VOD associated with worse 100-day OS. Therefore, intervention should be applied at the latest for moderate to severe SOS/VOD before deterioration.
传统的窦状隙阻塞综合征/静脉阻塞性疾病(SOS/VOD)严重程度标准是回顾性确定的,但在治疗决策中并不适用。根据改良的西雅图诊断标准,收集了 203 例 SOS/VOD 患者的数据,并对最近欧洲血液和骨髓移植学会(EBMT)提出的修订严重程度标准进行了验证分析。根据传统的严重程度标准,没有患者为轻度,而 63.1%为中度,36.9%为重度。然而,根据修订的 EBMT 标准,大多数患者(63.1%)为极重度,18.2%为重度,12.8%为中度,5.9%为轻度。轻度、中度、重度和极重度组的 100 天总生存率(OS)分别为 83.3%、84.3%、94.6%和 58.6%。极重度 SOS/VOD 的 OS 明显低于其他组(58.6 与 89.3%,p<0.0001)。整个队列的 100 天移植相关死亡率为 25.2%;轻度为 8.3%,中度为 8.0%,重度为 2.7%,极重度为 36.7%(p<0.0001)。修订后的 EBMT 标准新分类的极重度占与较差 100 天 OS 相关的 SOS/VOD 的大多数。因此,在恶化之前,应最晚对中度至重度 SOS/VOD 进行干预。