肝移植术后肝窦阻塞综合征的单中心诊疗经验

刘颖; 孙丽莹; 朱志军; 魏林; 曲伟; 曾志贵; 赵新颜

doi:10.3969/j.issn.1674-7445.2021.01.014

肝移植术后肝窦阻塞综合征的单中心诊疗经验

doi: 10.3969/j.issn.1674-7445.2021.01.014

100050 首都医科大学附属北京友谊医院肝脏移植中心（刘颖、朱志军、魏林、曲伟、曾志贵），重症医学科（孙丽莹），肝病中心（赵新颜），国家消化系统疾病临床医学研究中心（刘颖、孙丽莹、朱志军、魏林、曲伟、曾志贵、赵新颜）

基金项目:

首都临床特色应用研究 Z181100001718220

详细信息

作者简介:
刘颖，女，1981年生，博士，副主任医师，研究方向为移植感染与免疫，Email：xiaodouzai@hotmail.com

通讯作者:
孙丽莹，女，1967年生，博士，主任医师，研究方向为肝移植及肝病患者的重症管理、移植感染与免疫，Email：sunxlx@outlook.com

中图分类号: R617
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出版历程
- 收稿日期: 2020-09-10
- 网络出版日期: 2021-01-19
- 刊出日期: 2021-01-19

Diagnosis and treatment of hepatic sinusoidal obstruction syndrome after liver transplantation: a single-center experience

Liver Transplantation Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China

More Information

Corresponding author: Sun Liying, E-mail:sunxlx@outlook.com

摘要

摘要: 目的总结肝移植术后肝窦阻塞综合征（HSOS）的发病情况和诊疗经验。方法回顾性分析4例肝移植术后HSOS患者的临床资料。收集肝移植术后HSOS的发病情况、临床表现、影像学及病理学特点，分析HSOS患者的治疗方式及转归情况。结果肝移植术后HSOS的发生率为0.8%（2/239），中位发病时间为肝移植术后4.5（1.7，9.0）个月。HSOS的临床表现主要包括腹胀、腹腔积液、肝肿大、胆红素升高，部分伴有肾功能不全。4例HSOS患者的腹部增强CT均呈“花斑样”不均匀强化；肝组织病理学表现主要为肝窦扩张伴淤血。4例患者均给予调整免疫抑制剂，将他克莫司（Tac）转换为环孢素，并加用华法林抗凝治疗，其中1例患者接受经颈静脉肝内门体静脉分流术（TIPS）治疗。3例患者经治疗后症状完全缓解，1例患者治疗无效死亡，1例患者在治疗好转后因肺部感染和消化道出血死亡。结论 HSOS是肝移植术后少见但致命的并发症，及时的诊断和治疗可以避免移植物衰竭，改善患者预后。
- 肝窦阻塞综合征 /
- 肝移植 /
- 药物性肝损伤 /
- 抗凝治疗 /
- 腹腔积液 /
- 经颈静脉肝内门体静脉分流术 /
- 他克莫司 /
- 抗心磷脂抗体
Abstract: Objective To summarize the incidence, diagnostic and therapeutic experience of hepatic sinusoidal obstruction syndrome (HSOS) after liver transplantation. Methods Clinical data of 4 patients with HSOS after liver transplantation were retrospectively analyzed. The incidence, clinical manifestations, imaging and pathological characteristics of HSOS after liver transplantation were collected, and the treatment methods and clinical outcomes of patients with HSOS were analyzed. Results The incidence of HSOS after liver transplantation was 0.8%(2/239), and the median time of onset was 4.5(1.7, 9.0) months after liver transplantation. The clinical manifestations of HSOS mainly included abdominal distension, ascites, hepatomegaly, increased bilirubin, and renal insufficiency in partial cases. Enhanced abdominal CT scan of 4 patients with HSOS showed uneven spot-like enhancement and the liver histopathological examination mainly showed the signs of hepatic sinusoidal dilatation complicated with congestion. Four patients were administered with an adjusted regime of immunosuppressant by replacing tacrolimus (Tac) with ciclosporin and adding anticoagulant therapy with warfarin. One patient received transjugular intrahepatic portosystemic shunt (TIPS). After treatment, the symptoms of 3 patients were completely relieved, and 1 patient died. One of the 3 surviving patients died from pulmonary infection and gastrointestinal bleeding. Conclusions HSOS is a rare and fatal complication after liver transplantation. Timely diagnosis and treatment can avoid the incidence of graft failure and improve clinical prognosis of the patients.
- Hepatic sinusoidal obstruction syndrome /
- Liver transplantation /
- Drug-induced liver injury /
- Anticoagulant therapy /
- Ascites /
- Transjugular intrahepatic portosystemic shunt /
- Tacrolimus /
- Anticardiolipin antibody

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图 1 肝移植术后HSOS患者治疗前后的腹部增强CT图像

注：A图示治疗前移植肝肿大，伴不均匀强化，呈“花斑样”；B图示治疗后移植肝肿大和不均匀强化明显缓解。

Figure 1. Enhanced CT images of the abdomen before and after treatment in a patient with HSOS after liver transplantation

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图 2 肝移植术后HSOS患者治疗前后的肝组织病理学表现（Masson，×200）

注：A图示治疗前肝窦扩张伴有淤血；B图示治疗后肝窦淤血消失，肝窦扩张明显缓解。

Figure 2. Histopathological findings of liver tissues before and after treatment in a patient with HSOS after liver transplantation

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表 1 4例肝移植术后HSOS患者的临床资料

Table 1. Clinical data of 4 patients with HSOS after liver transplantation

例序	性别	年龄（岁）	原发病	术后发病时间（月）	临床表现	影像学表现	病理学表现	治疗	是否好转	预后	死因
1	男	47	药物性肝损伤	9.0	腹胀、肝功能异常、双下肢水肿、腹腔积液、肾损伤	CT：移植肝肿大，强化不均，腹腔积液	肝小静脉闭塞	Tac转换为环孢素，加用吗替麦考酚酯+甲泼尼龙	是	死亡	肺部感染、消化道出血
2	女	47	原发性胆汁淤积性肝硬化	1.7	腹腔积液、腹胀、肝肿大、胆红素轻度升高	超声：移植肝回声欠均匀，大量腹腔积液；CT：移植肝强化不均，腹腔积液	肝窦扩张、淤血	Tac转换为环孢素；华法林抗凝	是	痊愈	-^①
3	女	64	上皮样血管内皮细胞瘤	7.0	腹胀纳差、肝功能异常、肾功能不全	超声：移植肝增大并实质回声不均，大量腹腔积液；CT：移植肝肿大，强化不均，腹腔积液	肝窦扩张、淤血，伴肝细胞坏死	停用Tac；TIPS；华法林抗凝	否	死亡	消化道出血、移植肝衰竭
4	女	28	肝移植术后HSOS复发	2.0	肝功能异常、肝肿大、腹胀、腹腔积液、肾损伤	超声：移植肝形态饱满，回声不均，腹腔积液；CT：移植肝肿大，强化不均，腹腔积液	肝窦扩张、淤血	Tac转换为环孢素；华法林抗凝	是	痊愈	-
注：①-为无数据。

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表 2 肝移植术后HSOS患者治疗前后的肝肾功能变化

Table 2. Changes of liver and kidney function before and after treatment in patients with HSOS after liver transplantation

例序	ALT（U/L）		AST（U/L）		Scr（μmol/L）		DB（μmol/L）		TB（μmol/L）
例序	治疗前	治疗后	治疗前	治疗后	治疗前	治疗后	治疗前	治疗后	治疗前	治疗后
1	34	18	51	34	122	82	99	22	219	30
2	23	10	15	15	67	52	16	4	41	12
3	309	-^①	174	-	260	-	32	-	43	-
4	39	9	61	17	130	64	24	12	40	26
注：①-为无数据。

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