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目的探讨血色素沉积症(HC)的临床病理特点,为临床诊治提供依据。方法选择行肝穿刺确诊HC患者20例,肝组织行HE和组织化学染色,进行临床病理分析。结果原发性血色素沉积症(HHC)和继发性血色素沉积症(SHC)各10例。最常见的临床表现为乏力(18/20)、肝大(18/20)、脾大(17/20)。依据含铁血黄素沉积模式不同,HHC型17例,非HHC型3例。10例HHC患者均为HHC型,而10例SHC患者中,7例为HHC型,3例为非HHC型。HHC特征性的组织病理学表现是肝组织内含铁血黄素沉积及纤维化。脂肪变性、嗜酸性粒细胞浸润及空泡状的糖原核在肝组织内也较易见,且其分布区域与含铁血黄素沉积一致。统计学分析显示,HHC患者的肝纤维化程度与肝组织内铁沉积程度、血清铁呈正相关(P<0.05);而肝纤维化程度与病程、肝细胞脂肪变性程度无相关性(P>0.05)。SHC患者的肝纤维化程度与肝组织内铁沉积程度、血清铁、病程及肝细胞脂肪变性程度无相关性(P>0.05)。结论肝组织病理学检查是诊断HC的主要依据,结合病史和实验室检查,可以鉴别HHC及SHC。HHC可能是一类因铁过载打破机体内环境的稳定,损伤多器官功能结构的代谢疾病。故HHC应早诊断早治疗,避免出现多器官功能衰竭。
Objective To investigate the clinicopathological characteristics of hemochromatosis (HC) and provide the basis for clinical diagnosis and treatment. Methods Twenty patients diagnosed as HC by liver biopsy were selected. The liver tissues were stained with HE and histopathology, and analyzed clinically and pathologically. Results There were 10 cases of primary hemochromatosis (HHC) and secondary hemochromatosis (SHC). The most common clinical manifestations of fatigue (18/20), hepatomegaly (18/20), splenomegaly (17/20). According to different hemosiderin deposition patterns, HHC type 17 cases, non-HHC type 3 cases. Of the 10 HHC patients, all were HHC, and of the 10 SHC patients, 7 were HHC and 3 were non-HHC. HHC characteristic histopathological manifestations of hepatic hemosiderin deposition and fibrosis. Steatosis, eosinophilic infiltration and vacuolar glycogen nuclei are also more readily seen in liver tissue, and their distribution is consistent with hemosiderin deposition. Statistical analysis showed that the degree of hepatic fibrosis in HHC patients was positively correlated with the degree of iron deposition in liver tissue and serum iron (P <0.05), while there was no correlation between the degree of hepatic fibrosis and the course of disease and the degree of fatty degeneration of hepatocytes (P> 0.05 ). The degree of hepatic fibrosis in SHC patients was not related to the degree of iron deposition in liver tissue, serum iron, duration of disease and degree of steatosis in hepatocytes (P> 0.05). Conclusion Liver histopathology is the main basis for the diagnosis of HC, combined with history and laboratory tests, can identify HHC and SHC. HHC may be a type of metabolic disease that breaks down the internal environment of the body due to iron overload and damages the structure of multiple organ functions. Therefore, early diagnosis and treatment of HHC should be early to avoid multiple organ failure.