肝脏轻链沉积病与轻链型淀粉样变性的临床病理特征

Clinical pathological characteristics of light chain deposition disease and light chain amyloidosis in liver

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 explore the clinical pathological characteristics and the differential diagnosis of liver light chain deposition disease （LCDD）and light-chain amyloidosis （AL）. Methods Liver biopsies from 2012 to 2016 in Beijing You＇ an Hospital were retrospectively analyzed. Eight hepatic amyloidosis cases were diagnosed. Congo red stain, polarized light microscopy and immunohistochemical detection of light chain protein K, k were used. Then the clinical pathological characteristics were analyzed. Results All the patients were male. Hepatomegaly, increased GGT and ALP, different levels of proteinuria were the prominent clinical manifestations. Six cases were AL, and the other 2 cases were LCDD. Congo red stain was positive in the sinusoids in 6 AL cases, in which 2 cases with vessel wall positive, 4 cases with light chain protein K deposition, 2 cases with light chain protein k deposition. Congo red stain was negative in 2 LCDD cases, with light chain k protein deposition. Conclusion It is not easy to identify liver LCDD and AL from other primary liver diseases through clinical manifestations because that liver LCDD and AL lack clinical specificity. The diag- nosis of these two diseases relies on Congo red stain and light chain protein detection. So when there is clinical suspect of liver amyloid lesion,the patients should undergo liver biopsies in order to make clear diagnosis as early as possible.