12例肝结节状再生性增生临床分析

Nodular regenerative hyperplasia of the liver:a clinical study of 12 cases

目的对肝结节状再生性增生(NRHL)的临床、病理及诊断分析总结,期望提高临床医师对本病的认识。方法从300例因脾功能亢进而行脾切除和肝脏活组织检查病例中选出病理诊断符合NRHL 的病例12例,分析其病史、临床症状和体征、实验室检查、诊断及处理等资料,且随访治疗效果。结果12例 NRHL 中6例明确诊断为系统性红斑狼疮,1例克罗恩病,1例疑诊溃疡性结肠炎。应用肾上腺皮质激素治疗6例,免疫抑制剂3例。11例有门脉高压;所有患者均无病毒性肝炎史;肝功能轻度受损;病理特征为肝实质内结节状再生性增生,门脉周围轻度纤维化和汇管区散在淋巴细胞浸润,门静脉分支狭窄和闭塞,无肝坏死表现;术前均被诊断为肝硬化伴门脉高压;行手术治疗后临床症状明显缓解,随访患者多数病情稳定。结论 NRHL 可能与免疫和肝脏血液循环障碍有关;以门脉高压为主要表现,应与肝硬化鉴别;诊断依靠肝脏楔形活检;处理门脉高压可使临床状况得到改善。

Objective To review the clinical, histological and diagnostic aspects of 12 documented cases of nodular regenerative hyperplasia of the liver（ NRHL）, to make this condition be understood and dealt with better. Method Twelve NRHL cases were diagnosed based on liver biopsy from 300 portal hypertension patients who had been underwent splenectomy. Imaging studies were performed as part of the diagnostic evaluation. Clinical manifestation and biochemical tests were recorded at the time of diagnosis. Management and prognosis were also reviewed. Results Most patients were complicated with autoimmune disease, 6 cases were diagnosed systemic lupus erythematesns and 1 was Crohn＇ s disease and 1 suspected ulcerative colitis. Six cases were treated by prednisone and 3 cases by immunosuppressant. Eleven cases had suffered from portal hypertension. All cases had no history of viral hepatitis. Biochemical tests showed mild increase of liver enzyme and relative normal synthetic liver function. The histological finding was nodular in the hepatic parenchyma, with mild poriportal fibrosis, intraportal lymphocytic infiltration, narrow and obstruction of branch of portal vein, and lack of hepatocyte necrosis..MI cases were diagnosed liver cirrhosis and portal hypertension before operation. Management was directed to portal hypertension and varices bleeding with satisfactory results. Most of them keep a stable condition during the follow-up. Conclusion The NRHL was uncommon and its cause and pathogenesis was unclear, may be related with immune and hepatic blood circulation disorder. It should be considered in patients with unexplained portal hypertension and distinguished it from liver cirrhosis. Liver biopsy confirms the diagnosis. Management directed to portal hypertension may improve clinical condition.