一例在符合诊断标准之前经组织学诊断的晚发性窦道阻塞综合征病例。

IF 1.7 4区 医学 Q3 HEMATOLOGY International Journal of Hematology Pub Date : 2025-01-05 DOI:10.1007/s12185-024-03909-7
Takashi Kuroha, Tatsuo Furukawa, Seiichi Yoshikawa, Akane Kaihatsu, Hiroki Nemoto, Hiroyuki Usuda, Toshio Yano
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引用次数: 0

摘要

窦状静脉阻塞综合征(SOS),又称肝静脉闭塞性疾病(VOD),是造血干细胞移植的一种危及生命的并发症。严重者,SOS/VOD发展为多器官衰竭,死亡率高达80%以上。基于严重程度评估的早期诊断和治疗改善了严重SOS/VOD的预后,但传统的诊断标准可能不足以早期诊断。我们在此报告一例严重的迟发性SOS/VOD,在临床表现明显之前已被组织学证实。尽管在满足临床诊断标准之前就开始了去纤维肽治疗,但疾病进展为多器官衰竭。本病例提示,在出现腹水、体重增加或高胆红素血症等临床表现之前,一些患者需要早期治疗,并支持建立预测SOS/VOD发病和严重程度的方法的必要性。
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A case of late-onset sinusoidal obstruction syndrome diagnosed histologically before meeting diagnostic criteria.

Sinusoidal obstruction syndrome (SOS), also known as hepatic veno-occlusive disease (VOD), is a life-threatening complication of hematopoietic stem cell transplantation. In severe cases, SOS/VOD progresses to multiple organ failure with a mortality rate higher than 80%. Early diagnosis and treatment based on severity assessment improve the prognosis of severe SOS/VOD, but conventional diagnostic criteria may be insufficient for an early diagnosis. We herein report a case of severe late-onset SOS/VOD that was histologically proven before clinical findings become evident. Although defibrotide therapy was started before the clinical diagnostic criteria were met, the disease progressed to multiple organ failure. This case suggests that some patients require earlier treatment before the appearance of clinical findings such as ascites and weight gain or hyperbilirubinemia, and supports the need to establish methods for predicting the onset and severity of SOS/VOD.

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来源期刊
CiteScore
3.90
自引率
4.80%
发文量
223
审稿时长
6 months
期刊介绍: The International Journal of Hematology, the official journal of the Japanese Society of Hematology, has a long history of publishing leading research in hematology. The journal comprises articles that contribute to progress in research not only in basic hematology but also in clinical hematology, aiming to cover all aspects of this field, namely, erythrocytes, leukocytes and hematopoiesis, hemostasis, thrombosis and vascular biology, hematological malignancies, transplantation, and cell therapy. The expanded [Progress in Hematology] section integrates such relevant fields as the cell biology of stem cells and cancer cells, and clinical research in inflammation, cancer, and thrombosis. Reports on results of clinical trials are also included, thus contributing to the aim of fostering communication among researchers in the growing field of modern hematology. The journal provides the best of up-to-date information on modern hematology, presenting readers with high-impact, original work focusing on pivotal issues.
期刊最新文献
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