Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Cell Biochem Biophys. 2012 Nov;64(2):115-21. doi: 10.1007/s12013-012-9379-5.
Nodular regenerative hyperplasia (NRH) of liver may be one of the leading causes of non-cirrhotic intrahepatic portal hypertension (NCIPH), although the exact relationship is currently unknown. Diagnosis of NRH is relatively difficult and involves surgical pathology, and thus it is necessary to improve the preoperative recognition of NRH. Here, we analyze 15 cases of NRH to better understand this disease. All the liver specimens were microscopically examined by hematoxylin-eosin staining and reticulin and Masson trichrome staining. Diagnoses of NRH were confirmed by pathological examination. Clinically, NRH presents as diffused liver lesions with mildly increased liver enzymes. Portal hypertension is the most common clinical manifestation presenting prominently as splenomegaly, hypersplenism, and esophageal varices bleeding. NRH is often associated with autoimmune or collagen vascular diseases, and such patients often present with a variety of positive autoantibodies and increased erythrocyte sedimentation rate (ESR), Ig and γ %. Pathological examination of the liver showed diffuse small regenerative nodules without fibrous septa and obstructive portal venopathy. For those patients with portal hypertension of unknown cause and preserved liver function, especially, those combined with autoimmune diseases, NRH should be considered.
肝脏结节性再生性增生 (NRH) 可能是无肝硬化性肝内门静脉高压症 (NCIPH) 的主要原因之一,尽管确切的关系目前尚不清楚。NRH 的诊断相对困难,涉及手术病理学,因此有必要提高对 NRH 的术前认识。在这里,我们分析了 15 例 NRH 病例,以更好地了解这种疾病。所有肝脏标本均通过苏木精-伊红染色、网状纤维和 Masson 三色染色进行显微镜检查。通过病理检查确诊为 NRH。临床上,NRH 表现为弥漫性肝脏病变,肝酶轻度升高。门静脉高压症是最常见的临床表现,主要表现为脾肿大、脾功能亢进和食管静脉曲张出血。NRH 常与自身免疫或胶原血管疾病相关,此类患者常伴有多种自身抗体阳性和红细胞沉降率 (ESR)、Ig 和γ%升高。肝脏的病理检查显示弥漫性小结节性再生,无纤维间隔和阻塞性门静脉病变。对于那些原因不明的门静脉高压症和肝功能正常的患者,特别是那些伴有自身免疫性疾病的患者,应考虑 NRH。