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异基因干细胞移植受者迟发性严重肝窦阻塞综合征:一例报告

Late-onset severe hepatic sinusoidal obstruction syndrome in an allogeneic stem cell transplant recipient: A case report.

作者信息

Sun Yan-Ling, Liu Ling-Ling, He Yi, Zhang Jing-Wen, Wen Rui-Juan, Yuan Qing, Wang Xin, Guo Ruo-Mi, Li Xu-Dong, Long Bing

机构信息

Department of Hematology.

Department of Blood Transfusion, The Third Affiliated Hospital, Sun Yat-sen University.

出版信息

Medicine (Baltimore). 2020 Oct 23;99(43):e22927. doi: 10.1097/MD.0000000000022927.

Abstract

RATIONALE

Hepatic sinusoidal obstruction syndrome (SOS) is a rare and potentially fatal complications after hematopoietic stem cell transplantation (HSCT). Most severe SOS result in multi-organ dysfunction and are associated with a high mortality rate (>80%).

PATIENT CONCERNS

A 31-year-old man was diagnosed with chronic myeloid leukemia blast crisis. He presented with severe thrombocytopenia on day 42 post-HSCT (on days +42), gradually developed with painful hepatomegaly, ascites, and weight gain.

DIAGNOSES

The abdominal computerized tomography showed hepatomegaly, hepatic congestion, periportal edema, narrow hepatic vein, and ascites suggestive of SOS/hepatic vein occlusion. According to the EBMT revised diagnostic criteria, the patient was diagnosed as late-onset severe SOS.

INTERVENTIONS

Comprehensive treatment including low molecular weight heparin was initiated.

OUTCOMES

The patient had good response with resolution of his hepatomegaly, increase of platelet, weight and transaminase loss after 4 weeks treatment.

LESSONS

In SOS patients with nonspecific clinical and biochemical findings, computerized tomography scans can be useful in differentiating SOS from other complications after HSCT. low molecular weight heparin is effective for the treatment of SOS.

摘要

理论依据

肝窦阻塞综合征(SOS)是造血干细胞移植(HSCT)后一种罕见且可能致命的并发症。大多数严重的SOS会导致多器官功能障碍,并伴有高死亡率(>80%)。

患者情况

一名31岁男性被诊断为慢性髓性白血病急变期。他在HSCT后第42天(+42天)出现严重血小板减少,随后逐渐出现肝肿大、疼痛、腹水和体重增加。

诊断

腹部计算机断层扫描显示肝肿大、肝充血、门静脉周围水肿、肝静脉狭窄和腹水,提示SOS/肝静脉阻塞。根据欧洲血液与骨髓移植协会(EBMT)修订的诊断标准,该患者被诊断为迟发性严重SOS。

干预措施

开始包括低分子量肝素在内的综合治疗。

结果

经过4周治疗,患者肝肿大消退、血小板增加、体重减轻且转氨酶下降,反应良好。

经验教训

对于临床表现和生化检查结果不具特异性的SOS患者,计算机断层扫描有助于将SOS与HSCT后的其他并发症区分开来。低分子量肝素对SOS治疗有效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ac3/7581065/f6121cfec5d8/medi-99-e22927-g001.jpg

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