Clinicopathological features of hepatic amyloidosis:an analysis of 8 cases
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摘要:
目的探讨肝淀粉样变性的临床病理特征。方法收集解放军第三〇二医院2005年1月-2012年12月行肝穿刺活组织确诊的8例肝淀粉样变性患者,回顾性分析其临床资料、实验室检查、影像学以及病理学相关资料,归纳肝淀粉样变性的临床病理特点。结果临床表现及体征:肝肿大(7/8)、乏力(6/8)、纳差(6/8)、脾肿大(4/8)、腹水(2/8)、门静脉高压(2/8)、双下肢水肿(2/8)、皮肤瘙痒(2/8)、心脏病病史(2/8)、静脉曲张(1/8)。实验室检查:ALP升高(8/8)、GGT升高(5/8)、低蛋白血症(2/8)、ALT异常(2/8)、AST轻度升高(1/8)、TBil异常(1/8)。影像学检查:B超显示肝实质呈不均匀的回声图像(8/8)、瞬时弹性肝脏硬度检查显示明显增高(2/2)、CT显示弥散减弱(4/8)或局灶的减弱(2/8)。组织病理学和免疫病理学特点:HE染色在肝窦(8/8)及中央静脉周围(6/8)可见大量均染的粉红色样物质沉积,均染的粉红色物质刚果红染色为阳性(8/8),在偏振光显微镜下均染物质呈现苹果绿色,诊断为淀粉样变性;免疫组化Kappa染色阳性(6/8),Lamda染色阳...
Abstract:Objective To investigate the clinicopathological features of hepatic amyloidosis. Methods A retrospective analysis was performed for 8 patients with hepatic amyloidosis confirmed by liver biopsy in 302 Hospital of PLA from January 2005 to December 2012. Clinical data, laboratory examination results, imaging findings, and pathological data were analyzed to summarize the clinicopathological features of hepatic amyloidosis. Results The clinical manifestations and signs of this disease included hepatomegaly ( 7/8) , weakness ( 6/8) , poor appetite ( 6/8) , splenomegaly ( 4/8) , ascites ( 2/8) , portal hypertension ( 2/8) , edema of lower extremities ( 2/8) , pruritus ( 2/8) , a history of heart disease ( 2/8) , and varices ( 1/8) . Laboratory examination showed increases in alkaline phosphatase ( ALP) ( 8/8) and gamma-glutamyl transpeptidase ( 5/8) , hypoproteinemia ( 2/8) , abnormal alanine aminotransferase ( 2/8) , a slight increase in aspartate aminotransferase ( 1/8) , and abnormal total bilirubin ( 1/8) . As for imaging examinations, ultrasound showed uneven echo of liver parenchyma ( 8/8) ; transient elastography showed a significant increase in liver stiffness ( 2/2) ; CT showed diffuse weakness ( 4/8) or focal weakness ( 2/8) . As for histopathological and immunopathological features, HE staining showed the deposition of pink substances around the hepatic sinusoids ( 8/8) and the central vein ( 6/8) , Congo red staining of the pink substances obtained a positive result ( 8/8) , and the pink substances showed a color of apple green under a polarization microscope; therefore, the patients were diagnosed with amyloidosis. As for immunohistochemistry, of all patients, 6 had positive Kappa staining and 4 had positive Lamda staining. Conclusion Hepatomegaly and elevated ALP are relatively common in patients with primary hepatic amyloidosis. When a patient has hepatomegaly and elevated ALP and cannot be diagnosed with common liver diseases, clinical physicians should consider the possibility of hepatic amyloidosis, which is rare but fatal, and make a confirmed diagnosis based on related examinations, in order to provide guidance for clinical treatment.
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Key words:
- liver diseases /
- amyloidosis /
- disease attributes
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