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目的通过对肝静脉闭塞病(HVOD)的临床和病理特点研究,提高对该病的认识。方法回顾性分析因骨髓移植和服用土三七所致12例HVOD的临床和病理特征。结果骨髓移植所致HVOD常表现为急性起病,且病情较重;而土三七所致HVOD除急性起病外,也可以表现为亚急性、慢性起病,且病情轻重不一,可能与土三七的剂量和疗程以及个体差异相关。抗凝疗法治疗急性和亚急性HVOD疗效优于慢性HVOD。两者所致HVOD的病理均表现为肝窦扩张、瘀血,肝索挤压、萎缩,肝细胞坏死,胆汁淤积,而肝小静脉缩窄甚至闭塞等典型表现并不常见,可能与经皮肝穿刺活检术有很大局限性有关。结论骨髓移植和应用土三七应警惕HVOD的发生,争取早期诊断和及时治疗。
Objective To improve the understanding of the disease by studying the clinical and pathological features of hepatic veno-occlusive disease (HVOD). Methods We retrospectively analyzed the clinical and pathological features of 12 patients with HVOD induced by bone marrow transplantation and taking Sanqi. Results The HVOD induced by bone marrow transplantation often showed acute onset and serious condition. Besides the acute onset of HVOD, the HVOD induced by bone marrow transplantation also showed subacute and chronic onset, and the severity of the disease may be related to Soil thirty-seven dose and treatment and individual differences related. Anticoagulant therapy is superior to chronic HVOD in the treatment of acute and sub-acute HVOD. The pathology of the HVOD caused by both showed sinusoidal dilatation, blood stasis, hepatic cord compression, atrophy, hepatocellular necrosis, cholestasis, and hepatic venules narrowing and occlusion and other typical performance is not common, may be associated with percutaneous Liver biopsy has great limitations related. Conclusion Bone marrow transplantation and the application of soil thirty-seven should be alert to the occurrence of HVOD, for early diagnosis and timely treatment.