低剂量去纤苷治疗吡咯生物碱相关肝窦阻塞综合征1例报告
DOI: 10.12449/JCH260421
Low-dose defibrotide in the treatment of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome: A case report
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摘要: 本文报道1例60岁男性因服“土三七”致吡咯生物碱相关肝窦阻塞综合征。患者表现为腹水及肝功能异常,经影像学及肝活检确诊。因患者对低分子肝素过敏且支持治疗无效,予低剂量去纤苷挽救治疗。治疗后腹水迅速消退、肝功能恢复,复查肝活检显示肝窦淤血减轻、肝细胞再生。治疗期间出现自愈性结膜出血。本病例提示,对于标准抗凝禁忌或无效者,低剂量去纤苷或为有效且相对安全的替代治疗方案。Abstract: This article reports a case of a male patient, aged 60 years, who developed pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome (PA-HSOS) due to ingestion of Gynura segetum (Lour.) Merr. The patient presented with ascites and abnormal liver function, and a confirmed diagnosis was made based on radiological examination and liver biopsy. Since the patient was allergic to low-molecular-weight heparin and had no response to supportive therapy, low-dose defibrotide was administered as rescue treatment. After treatment, the patient achieved rapid regression of ascites and recovery of liver function, and liver biopsy reexamination showed alleviation of sinusoidal congestion and hepatocyte regeneration. Self-resolving conjunctival hemorrhage occurred during treatment. This case suggests that for patients with contraindications to standard anticoagulation therapy or those showing no response to such treatment, low-dose defibrotide may be used as an effective and relatively safe alternative treatment regimen.
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注: a、b,苏木精-伊红染色;c、d,马松染色。初次肝脏病理学显示(a、c),中央静脉周围肝窦明显扩张,淤血性肝细胞萎缩,部分肝细胞脱失,提示静脉回流障碍,符合HSOS。第2次肝脏病理学显示(b、d),Ⅲ带肝细胞再生性增生,窦细胞增生,局灶肝窦轻度扩张淤血;与初次肝穿刺比较,中央区肝窦淤血明显减轻,坏死区肝细胞大部分再生,符合急性HSOS修复期改变。HSOS,肝窦阻塞综合征。
图 2 去纤苷治疗前后肝脏病理学变化(×200)
Figure 2. Pathological comparison of liver biopsy before and after use of defibrotide (×200)
表 1 去纤苷治疗前后肝功能及其他指标的变化
Table 1. Changes in liver function and other clinical indicators before and after defibrotide treatment
检查时间 ALT(U/L) AST(U/L) TBil(μmol/L) TBA(μmol/L) GGT(U/L) Alb(g/L) PLT(×109/L) PT(s) INR 2024/1/12 24.1 40.2 46.9 46.9 239.3 43.5 106 10.0 1.13 2024/1/26 30.2 35.1 26.9 40.0 117.3 40.1 109 9.9 1.13 2024/2/10 16.2 23.4 10.2 7.3 98.1 36.1 143 9.5 1.08 2024/2/18 16.3 23.3 10.2 5.0 128.1 37.3 143 8.8 1.00 2024/2/29 21.3 27.3 8.9 4.9 76.2 36.6 144 9.8 1.12 注:ALT,丙氨酸氨基转移酶;AST,天门冬氨酸氨基转移酶;TBil,总胆红素;TBA,总胆汁酸;GGT,γ-谷氨酰转移酶;Alb,白蛋白;PLT,血小板计数;PT,凝血酶原时间;INR,国际标准化比值。
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