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回顾性应用窦阻塞综合征/静脉阻塞病诊断标准于儿科造血干细胞移植队列。

Retrospective Application of Sinusoidal Obstruction Syndrome/Veno-occlusive Disease Diagnostic Criteria in a Pediatric Hematopoietic Stem Cell Transplant Cohort.

机构信息

Division of Pediatric Hematology and Oncology, University of Miami Miller School of Medicine, Miami, FL.

Division of Pediatric Hematology, Oncology and Blood and Marrow Transplant.

出版信息

J Pediatr Hematol Oncol. 2022 Mar 1;44(2):e343-e348. doi: 10.1097/MPH.0000000000002267.

Abstract

Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) posthematopoietic stem cell transplantation (HSCT) is often diagnosed using the modified Seattle (MS) or European Society for Blood and Marrow Transplantation (EBMT) criteria. We hypothesized that strict application of these criteria could affect the timing of diagnosis and incidence of SOS/VOD. We collected data on 215 transplants performed in 184 patients at a single pediatric hematopoietic stem cell transplantation center, which were divided into 3 cohorts. Clinical diagnosis and treatment of SOS/VOD was documented in 13% of transplants (cohort 1). On retrospective review, 49% of transplant events met either MS and/or EBMT criteria, however, were not diagnosed with SOS/VOD (cohort 2); remaining 38% of transplant events did not meet MS or EBMT criteria and were not diagnosed with SOS/VOD (cohort 3). Day+100 overall survival was significantly inferior for cohort 1 (78%) compared with cohorts 2 or 3 (92% and 95%, P=0.01) with no difference between cohorts 2 and 3 (P=0.5). Patients diagnosed with SOS/VOD >day+13 had worse day+100 overall survival when compared with those diagnosed ≤day13 (64.3% and 100%, respectively, P=0.02). This study highlights the value of careful clinical assessment to guide diagnosis and the need to refine diagnostic criteria for SOS/VOD in children.

摘要

造血干细胞移植(HSCT)后发生的窦状隙阻塞综合征/静脉阻塞病(SOS/VOD)通常采用改良西雅图(MS)或欧洲血液和骨髓移植学会(EBMT)标准进行诊断。我们假设严格应用这些标准可能会影响 SOS/VOD 的诊断时间和发生率。我们收集了单儿科造血干细胞移植中心 184 名患者 215 例移植的数据,将其分为 3 组。在 13%的移植中(第 1 组)记录了 SOS/VOD 的临床诊断和治疗。回顾性分析显示,49%的移植事件符合 MS 和/或 EBMT 标准,但未诊断为 SOS/VOD(第 2 组);其余 38%的移植事件不符合 MS 或 EBMT 标准且未诊断为 SOS/VOD(第 3 组)。第 1 组的 100 天总生存率(78%)明显低于第 2 组或第 3 组(92%和 95%,P=0.01),第 2 组和第 3 组之间无差异(P=0.5)。与第 13 天之前诊断为 SOS/VOD 的患者相比,第 13 天之后诊断为 SOS/VOD 的患者 100 天总生存率更差(分别为 64.3%和 100%,P=0.02)。本研究强调了仔细临床评估指导诊断的价值,并需要对儿童 SOS/VOD 的诊断标准进行细化。

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