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Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a refined classification from the European society for blood and marrow transplantation (EBMT).成年患者肝窦阻塞综合征/静脉闭塞性疾病的诊断和严重程度标准:欧洲血液与骨髓移植学会(EBMT)的细化分类
Bone Marrow Transplant. 2023 Jul;58(7):749-754. doi: 10.1038/s41409-023-01992-8. Epub 2023 Apr 24.
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Innovative strategies to improve hematopoietic stem cell transplant outcomes in myelofibrosis.创新策略改善骨髓纤维化患者造血干细胞移植的结局。
Am J Hematol. 2022 Nov;97(11):1464-1477. doi: 10.1002/ajh.26654. Epub 2022 Jul 19.
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Impact of prior JAK-inhibitor therapy with ruxolitinib on outcome after allogeneic hematopoietic stem cell transplantation for myelofibrosis: a study of the CMWP of EBMT.既往使用鲁索利替尼治疗 JAK 抑制剂对骨髓纤维化患者异基因造血干细胞移植后结局的影响:来自 EBMT 的 CMWP 的研究。
Leukemia. 2021 Dec;35(12):3551-3560. doi: 10.1038/s41375-021-01276-4. Epub 2021 May 22.
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Predicting sinusoidal obstruction syndrome after allogeneic stem cell transplantation with the EASIX biomarker panel.用 EASIX 生物标志物面板预测异基因干细胞移植后的窦房结阻塞综合征。
Haematologica. 2021 Feb 1;106(2):446-453. doi: 10.3324/haematol.2019.238790.
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3023 Mayo Clinic Patients With Myeloproliferative Neoplasms: Risk-Stratified Comparison of Survival and Outcomes Data Among Disease Subgroups.3023 例梅奥诊所骨髓增殖性肿瘤患者:疾病亚组间生存和结局数据的风险分层比较。
Mayo Clin Proc. 2019 Apr;94(4):599-610. doi: 10.1016/j.mayocp.2018.08.022. Epub 2019 Feb 26.
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Risk Factors for Development of and Progression of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome.肝静脉闭塞病/窦状隙阻塞综合征发展和进展的风险因素。
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Clinical effects of recombinant thrombomodulin and defibrotide on sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell transplantation.重组血栓调节蛋白和去纤维肽对异基因造血干细胞移植后窦阻塞综合征的临床疗效。
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Low-dose splenic irradiation prior to hematopoietic cell transplantation in hypersplenic patients with myelofibrosis.
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Sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell transplantation: Incidence, risk factors and outcomes.异基因造血干细胞移植后的窦性阻塞综合征:发病率、危险因素及预后
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Splenic volume may be a useful indicator of the protective effect of bevacizumab against oxaliplatin-induced hepatic sinusoidal obstruction syndrome.脾脏体积可能是贝伐珠单抗预防奥沙利铂诱导的肝窦阻塞综合征的保护作用的一个有用指标。
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异基因造血干细胞移植前脾脏照射作为肝窦阻塞综合征的一个可能危险因素:三例报告

Splenic Irradiation Preceding Allogeneic Hematopoietic Stem Cell Transplantation as a Possible Risk Factor of Sinusoidal Obstruction Syndrome: A Report of Three Cases.

作者信息

Shirane Shuichi, Yasuda Hajime, Uchimura Ayana, Mori Yosuke, Inano Tadaaki, Tsutsui Miyuki, Hamano Yasuharu, Ando Miki

机构信息

Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.

出版信息

Case Rep Oncol. 2024 Dec 9;18(1):1-6. doi: 10.1159/000542608. eCollection 2025 Jan-Dec.

DOI:10.1159/000542608
PMID:39980503
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11627584/
Abstract

INTRODUCTION

Splenomegaly is frequently encountered in patients with myeloproliferative neoplasms. Splenomegaly is associated with an increased incidence of engraftment failure during allogeneic hematopoietic stem cell transplantation (allo-HSCT), and some centers perform prior low-dose splenic irradiation (LDSI) which has been reported to be both safe and effective. However, we report conflicting results by presenting three allo-HSCT patients undergoing LDSI that subsequently developed sinusoidal obstruction syndrome (SOS).

CASE PRESENTATION

The underlying diseases of the presented cases were atypical chronic myeloid leukemia, secondary myelofibrosis following essential thrombocythemia, and acute myeloid leukemia transforming from myeloproliferative neoplasm, unclassifiable. Endothelial Activation and Stress Index (EASIX) scores of the 3 patients were 0.40, 3.82, and 4.40, respectively.

CONCLUSION

SOS is a potentially fatal complication of allo-HSCT, and the mortality rates of severe cases are reported to be above 80%. LDSI has not been recognized as a risk factor of SOS to date. Alternative management methods of splenomegaly during allo-HSCT such as ruxolitinib administration may be safer compared to LDSI.

摘要

引言

脾肿大在骨髓增殖性肿瘤患者中经常出现。脾肿大与异基因造血干细胞移植(allo-HSCT)期间植入失败的发生率增加有关,一些中心会进行预先的低剂量脾照射(LDSI),据报道这种方法既安全又有效。然而,我们通过介绍3例接受LDSI后随后发生窦性阻塞综合征(SOS)的allo-HSCT患者,报告了相互矛盾的结果。

病例介绍

所呈现病例的基础疾病分别为非典型慢性髓性白血病、原发性血小板增多症继发的继发性骨髓纤维化以及无法分类的由骨髓增殖性肿瘤转化而来的急性髓性白血病。这3例患者的内皮激活和应激指数(EASIX)评分分别为0.40、3.82和4.40。

结论

SOS是allo-HSCT的一种潜在致命并发症,据报道严重病例的死亡率高于80%。迄今为止,LDSI尚未被认为是SOS的危险因素。与LDSI相比,allo-HSCT期间脾肿大的替代管理方法,如使用芦可替尼,可能更安全。