中华肝脏病杂志
中華肝髒病雜誌
중화간장병잡지
CHINESE JOURNAL OF HEPATOLOGY
2015年
6期
443-448
,共6页
孙磊%王鹏%张亮%滕晓英%周新刚%齐立明%郎振为%刘红刚
孫磊%王鵬%張亮%滕曉英%週新剛%齊立明%郎振為%劉紅剛
손뢰%왕붕%장량%등효영%주신강%제립명%랑진위%류홍강
血色素沉着症%肝脏%铁%病理学
血色素沉著癥%肝髒%鐵%病理學
혈색소침착증%간장%철%병이학
Hemochromatosis%Liver%Iron%Pathology
目的 探讨不同原因造成的血色病肝脏铁沉积程度、分布特点及其与组织学改变的关系.方法 对31例血色病患者肝活体组织标本进行HE、网状纤维、胶原纤维及普鲁士蓝染色,组织学改变及铁沉积情况分别用Ishak评分及Deugnier评分系统评估,部分病例留取外周血进行血色病及相关遗传代谢疾病基因检测.统计处理均使用SPSS 19.0统计软件,用t检验比较两组数据之间的差异. 结果 31例血色病中,1例为遗传性血色病基因突变的原发性血色病伴Gilbert综合征、4例Gilbert综合征铁沉积均主要位于Ⅰ区肝细胞内,围绕汇管区,Ⅰ区至Ⅲ区铁沉积递减,炎症及纤维化程度均较轻;1例Ⅰ型遗传性血色病基因突变的原发性血色病伴乙型肝炎铁沉积与8例病毒性肝炎铁沉积相似,均在小叶内弥漫分布,有明显的肝细胞、肝窦及汇管区细胞铁沉积,炎症及纤维化轻重不一;5例血液系统疾病铁沉积也主要位于Ⅰ区,但肝细胞及肝窦、汇管区细胞均有铁沉积,炎症及纤维化程度较轻;5例脂肪性肝病及5例药物性肝损伤铁沉积量较少,主要位于肿胀或气球样变的肝细胞内;2例食物中铁摄入过多,铁在小叶内弥漫分布,肝细胞、肝窦及汇管区细胞均有.非肝细胞铁沉积评分、肝脏炎症活动度评分和纤维化程度评分:有遗传性疾病患者和无遗传性疾病患者分别为4.09±4.01和8.75±6.54、2.45±1.13和8.20±4.15、0.91±0.30和2.70±1.38,t值分别是-2.45、-5.81、-5.57,P值分别<0.05 ~<0.01.结论 不同原因造成的血色病肝脏铁沉积模式不同,铁在肝内沉积程度、分布特点的分析有助于病因诊断及预后评估.
目的 探討不同原因造成的血色病肝髒鐵沉積程度、分佈特點及其與組織學改變的關繫.方法 對31例血色病患者肝活體組織標本進行HE、網狀纖維、膠原纖維及普魯士藍染色,組織學改變及鐵沉積情況分彆用Ishak評分及Deugnier評分繫統評估,部分病例留取外週血進行血色病及相關遺傳代謝疾病基因檢測.統計處理均使用SPSS 19.0統計軟件,用t檢驗比較兩組數據之間的差異. 結果 31例血色病中,1例為遺傳性血色病基因突變的原髮性血色病伴Gilbert綜閤徵、4例Gilbert綜閤徵鐵沉積均主要位于Ⅰ區肝細胞內,圍繞彙管區,Ⅰ區至Ⅲ區鐵沉積遞減,炎癥及纖維化程度均較輕;1例Ⅰ型遺傳性血色病基因突變的原髮性血色病伴乙型肝炎鐵沉積與8例病毒性肝炎鐵沉積相似,均在小葉內瀰漫分佈,有明顯的肝細胞、肝竇及彙管區細胞鐵沉積,炎癥及纖維化輕重不一;5例血液繫統疾病鐵沉積也主要位于Ⅰ區,但肝細胞及肝竇、彙管區細胞均有鐵沉積,炎癥及纖維化程度較輕;5例脂肪性肝病及5例藥物性肝損傷鐵沉積量較少,主要位于腫脹或氣毬樣變的肝細胞內;2例食物中鐵攝入過多,鐵在小葉內瀰漫分佈,肝細胞、肝竇及彙管區細胞均有.非肝細胞鐵沉積評分、肝髒炎癥活動度評分和纖維化程度評分:有遺傳性疾病患者和無遺傳性疾病患者分彆為4.09±4.01和8.75±6.54、2.45±1.13和8.20±4.15、0.91±0.30和2.70±1.38,t值分彆是-2.45、-5.81、-5.57,P值分彆<0.05 ~<0.01.結論 不同原因造成的血色病肝髒鐵沉積模式不同,鐵在肝內沉積程度、分佈特點的分析有助于病因診斷及預後評估.
목적 탐토불동원인조성적혈색병간장철침적정도、분포특점급기여조직학개변적관계.방법 대31례혈색병환자간활체조직표본진행HE、망상섬유、효원섬유급보로사람염색,조직학개변급철침적정황분별용Ishak평분급Deugnier평분계통평고,부분병례류취외주혈진행혈색병급상관유전대사질병기인검측.통계처리균사용SPSS 19.0통계연건,용t검험비교량조수거지간적차이. 결과 31례혈색병중,1례위유전성혈색병기인돌변적원발성혈색병반Gilbert종합정、4례Gilbert종합정철침적균주요위우Ⅰ구간세포내,위요회관구,Ⅰ구지Ⅲ구철침적체감,염증급섬유화정도균교경;1례Ⅰ형유전성혈색병기인돌변적원발성혈색병반을형간염철침적여8례병독성간염철침적상사,균재소협내미만분포,유명현적간세포、간두급회관구세포철침적,염증급섬유화경중불일;5례혈액계통질병철침적야주요위우Ⅰ구,단간세포급간두、회관구세포균유철침적,염증급섬유화정도교경;5례지방성간병급5례약물성간손상철침적량교소,주요위우종창혹기구양변적간세포내;2례식물중철섭입과다,철재소협내미만분포,간세포、간두급회관구세포균유.비간세포철침적평분、간장염증활동도평분화섬유화정도평분:유유전성질병환자화무유전성질병환자분별위4.09±4.01화8.75±6.54、2.45±1.13화8.20±4.15、0.91±0.30화2.70±1.38,t치분별시-2.45、-5.81、-5.57,P치분별<0.05 ~<0.01.결론 불동원인조성적혈색병간장철침적모식불동,철재간내침적정도、분포특점적분석유조우병인진단급예후평고.
Objective To identify the type of iron deposition and describe its amount,distribution and associated lesions,in order to support an etiologic diagnosis for hemochromatosis.Methods Hematoxylineosin (HE) stain,reticular fiber stain,Masson's stain and Perl's iron stain were used to assess liver biopsies from 31 patients with hemochromatosis.The Ishak scoring system and Deugnier scoring system were used to assess the histological change in liver and to semi-quantify the excess of hepatic iron.Genetic testing results were received from a portion of the patients and used in analysis.Results One patient had hereditary (-HFE) hemochromatosis complicated with Gilbert's syndrome,for which the pattern of iron deposition was similar to that of the four patients with Gilbert's syndrome.Iron accumulation appeared as fine granules predominating at the biliary pole of cells and was distributed throughout the lobule with a decreasing gradient spanning from the periportal to centrolobular areas.Mild chronic inflammation was found to be commonly associated with low stage fibrosis.One patient had HFE hemochromatosis complicated with hepatitis B virus infection,and the pattern of iron deposition resembled that in the eight patients with viral hepatitis,wherein the deposition was mainly in the sinusoidal cells and/or portal macrophages.Histological grading and fibrosis staging differed among patients.The five patients with blood disordered showed iron accumulation mainly in the periportal hepatocytes,but mesenchymal iron deposits were also present.The grade of inflammation,as well as of fibrosis,was mild.The five patients with alcoholic disease and the five patients with drug-induced hepatitis showed hepatic iron deposition in swollen or ballooned hepatocytes.The two patients with excessive iron supply showed iron deposition localized within the parenchymal and mesenchymal cells.Conclusion Etiologic diagnosis of hemochromatosis relies on both the type of iron deposition and the nature of associated lesions.Liver biopsy is necessary for both diagnosis and prognosis.