Late-onset severe hepatic sinusoidal obstruction syndrome in an allogeneic stem cell transplant recipient: A case report.
Late-onset severe hepatic sinusoidal obstruction syndrome in an allogeneic stem cell transplant recipient: A case report.
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同种异体干细胞移植受者晚发严重肝窦阻塞综合征
DOI：
10.1097/md.0000000000022927
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发表时间：
2020-10-23
期刊：
影响因子：
1.6
通讯作者：
Long B
中科院分区：
文献类型：
--
作者：
Sun YL;Liu LL;He Y;Zhang JW;Wen RJ;Yuan Q;Wang X;Guo RM;Li XD;Long B
摘要依据：肝窦阻塞综合征（SOS）是造血干细胞移植（HSCT）后的一种罕见且潜在致命的并发症。大多数严重的SOS导致多器官功能障碍，并与高死亡率（>80%）相关。患者问题：一名31岁男性被诊断为慢性粒细胞白血病急变。他在HSCT后第42天（第+42天）出现重度血小板减少症，逐渐发展为疼痛性肝肿大、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/肝静脉闭塞。根据EBMT修订的诊断标准，患者被诊断为晚发性重度SOS。干预措施：开始包括低分子量肝素在内的综合治疗。成果：治疗4周后，患者反应良好，肝肿大消退，血小板增加，体重和转氨酶下降。经验教训：在SOS患者的非特异性临床和生化结果，计算机断层扫描可以是有用的，在HSCT后的其他并发症的SOS区分。低分子量肝素对SOS的治疗有效。
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.