From the Department of Pediatric Hematology/Oncology & Bone Marrow Transplantation Unit, School of Medicine, Altunizade Hospital, Acıbadem University, Istanbul, Turkey.
Exp Clin Transplant. 2022 Jul;20(7):680-686. doi: 10.6002/ect.2021.0475. Epub 2022 May 23.
Sinusoidal obstruction syndrome/venoocclusive disease is a significant complication of hematopoietic stem cell transplantation. Due to high mortality rates, new treatment strategies have been investigated. Here, we have presented outcomes of therapeutic plasma exchange performed on patients with sinusoidal obstruction syndrome/veno-occlusive disease.
Our study included 70 pediatric patients diagnosed with sinusoidal obstruction syndrome/veno-occlusive disease. Therapeutic plasma exchange procedures in patients were evaluated retrospectively.
There were 9 mild (12.9%), 9 moderate (12.9%), 21 severe (30%), and 31 very severe (44.2%) cases of sinusoidal obstruction syndrome/venoocclusive disease. Therapeutic plasma exchange was performed in 31 of the 70 study patients (59.6%). Moreover, 10/21 patients with severe (47.6%) and 21/31 patients with very severe (67.7%) disease underwent plasma exchange. Mean time from diagnosis of sinusoidal obstruction syndrome/venoocclusive disease to therapeutic plasma exchange initiation was 2.3 days. The 31 patients who received therapeutic plasma exchange had a total of 146 sessions. Overall survival rates at 100 days were 87.1% and 92.3% for patients who did and did not undergo therapeutic plasma exchange, respectively. When patients with mild and moderate disease who were not expected to undergo plasma exchange were excluded (n = 52), 100-day overall survival rates were 87.1% and 90.5% for those who did and did not undergo plasma exchange, respectively. When we compared severe versus very severe groups, no significant difference was found.
Plasmapheresis had no positive effect on survival. However, overall survival in all groups was higher than that in the literature, despite the high number of patients with severe and very severe disease. Interpretation of the results is limited by the retrospective nature of the study. Thus, prospective, randomized controlled trials with larger numbers of patients are necessary to investigate the role of therapeutic plasma exchange in patients with sinusoidal obstruction syndrome/veno-occlusive disease.
窦状隙阻塞综合征/静脉阻塞性疾病是造血干细胞移植的一种严重并发症。由于死亡率高，已研究了新的治疗策略。在这里，我们报告了对窦状隙阻塞综合征/静脉阻塞性疾病患者进行治疗性血浆置换的结果。
本研究纳入了 70 例诊断为窦状隙阻塞综合征/静脉阻塞性疾病的儿科患者。回顾性评估了患者的治疗性血浆置换程序。
9 例为轻度（12.9%），9 例为中度（12.9%），21 例为重度（30%），31 例为极重度（44.2%）。70 例研究患者中有 31 例（59.6%）进行了治疗性血浆置换。此外，21 例重度（47.6%）和 31 例极重度（67.7%）疾病患者中 10 例和 21 例接受了血浆置换。从窦状隙阻塞综合征/静脉阻塞性疾病诊断到开始治疗性血浆置换的平均时间为 2.3 天。接受治疗性血浆置换的 31 例患者共进行了 146 次治疗。未进行治疗性血浆置换患者的 100 天总生存率为 87.1%，进行治疗性血浆置换患者的 100 天总生存率为 92.3%。排除未预期进行血浆置换的轻度和中度疾病患者（n=52）后，进行和未进行血浆置换患者的 100 天总生存率分别为 87.1%和 90.5%。重度与极重度组之间无显著差异。
血浆置换对生存率无积极影响。然而，尽管严重和极重度疾病患者较多，但所有组的总生存率均高于文献报道。结果的解释受到研究回顾性的限制。因此，需要进行前瞻性、随机对照试验，纳入更多患者，以探讨治疗性血浆置换在窦状隙阻塞综合征/静脉阻塞性疾病患者中的作用。
