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Clinical and pathological analysis of 20 cases of hemochromatosis

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Author(s): Li LIANG | Jing-min ZHAO | Guang-de ZHOU | Lu XIONG | Wen-shu LI | Hai-bin WANG | Xiao-dong GUO | Yu-lai ZHAO | Shu-hong LIU | Li-xin WEI

Journal: Medical Journal of Chinese People's Liberation Army
ISSN 0577-7402

Volume: 36;
Issue: 1;
Start page: 75;
Date: 2011;
Original page

Keywords: liver; hemochromatosis; iron

ABSTRACT
Objective To investigate the clinical and pathological characteristics of hemochromatosis(HC),and provide references for HC diagnosis and treatment.Methods Liver specimens were obtained via needle biopsy from 20 cases of HC.Histological specimens were stained with haematoxylin eosin.Pathological changes of liver tissues were analyzed together with the clinical data.Results Ten cases of hereditary hemochromatosis(HHC) and 10 cases of secondary hemochromatosis(SHC) were randomly selected.Fatigue(18/20),hepatomegalia(18/20) and splenomegalia(17/20)were the common clinical manifestations.The 20 HC cases characterized by iron overload and fibrosis may be divided into HHC type(17 cases) and non-HHC type(3 cases) according to the region of iron deposition.All the 10 cases of HHC showed HHC type,while 7 of the 10 SHC cases showed HHC type,and the other 3 SHC cases showed non-HHC type.Steatosis,eosinophile granulocyte infiltration and vacuolus nucleus were also observed frequently in the liver tissues of HC,and their distribution coincided with the region of iron deposition.Statistically,fibrosis was significantly associated with iron deposition and serum iron in HHC patients(P < 0.05),but not associated with steatosis and duration of HHC.Additionally,fibrosis was not associated with iron deposition,serum iron,steatosis and duration of SHC in SHC patients.Conclusions The final diagnosis of HC depends mainly on histological changes in liver tissues.Meanwhile,it is necessary to distinguish HHC from SHC according to case history and biochemical detection.HHC might be a metabolic disease with multi-organ damage due to the disruption of homeostasis by iron overload.To avoid multi-organ failure,patients with HHC should be diagnosed and treated as early as possible.

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