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目的探讨轻链沉积病(light chain deposition disease,LCDD)和轻链型淀粉样变性(light chain amyloido-sis,AL)累及肝脏的临床病理特征及鉴别诊断。方法回顾性分析北京佑安医院2012-2016年行肝穿刺活体组织检查,光镜初步诊断为肝淀粉样变的8例患者资料,进一步行刚果红染色、偏振光显微镜观察和轻链蛋白κ、λ的免疫组化检测,并分析其临床病理特点。结果 8例怀疑肝淀粉样变的患者均为中老年男性,临床表现为肝脏肿大,γ-谷氨酰转肽酶和碱性磷酸酶升高为主的肝功能异常,以及不同程度的蛋白尿,其中6例证实为AL,2例为LCDD。6例AL刚果红染色肝窦内呈不同程度阳性,2例伴血管壁阳性,其中4例为轻链蛋白κ沉积,2例为轻链蛋白λ沉积。2例LCDD刚果红染色均阴性,且均为轻链蛋白λ沉积。结论以肝脏受累为突出表现的LCDD和AL,临床表现缺乏特异性,与肝脏原发疾病不易鉴别,本病确诊依赖刚果红染色及轻链蛋白检测,临床怀疑肝淀粉样病变时应尽早行肝穿刺活检以明确诊断。
Objective To investigate the clinicopathological characteristics and differential diagnosis of light chain deposition disease (LCDD) and light chain amyloido-sis (AL) in the liver. Methods The data of 8 patients with liver amyloidosis diagnosed by liver biopsy from 2012 to 2016 in Beijing You’an Hospital were analyzed retrospectively. The data of Congo red staining, polarized light microscopy and light chain kappa B, λ immunohistochemical detection, and analysis of its clinical and pathological features. Results Eight cases of patients with suspected hepatic amyloidosis were all middle-aged and old men. The clinical manifestations were liver dysfunction, hepatic dysfunction with enlarged liver, elevated γ-glutamyl transpeptidase and alkaline phosphatase, and various degrees of protein Urine, 6 cases confirmed as AL, 2 cases of LCDD. 6 cases of AL Congo red staining of sinusoidal sinus showed varying degrees of positive, 2 cases with vessel wall positive, of which 4 cases of light chain protein kappa deposition, 2 cases of light chain protein λ deposition. Two cases of LCDD Congo red staining were negative, and all light chain protein λ deposition. Conclusions The LCDD and AL, which are characterized by liver involvement, are not specific in clinical manifestations and are not easily distinguished from the primary liver disease. The diagnosis of this disease depends on detection of Congo red staining and light chain protein. Liver cirrhosis should be treated as soon as possible Puncture biopsy to confirm the diagnosis.