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Clinical and pathological features of liver tissues of patients with Gaucher’s disease

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Author(s): Yan-ling SUN | Jing-min ZHAO | Guang-de ZHOU | Li-xin WEI

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

Volume: 36;
Issue: 1;
Start page: 89;
Date: 2011;
Original page

Keywords: gaucher disease; pathology | clinical

ABSTRACT
Objective To study the clinical and pathological characteristics of Gaucher’s disease.Methods The clinical data of three patients with Gaucher’s disease were analyzed.The pathological morphology of Gaucher’s disease was observed under microscopy and double immunohistochemical staining was performed.Results All the 3 cases of Gaucher’s disease(including two females and one male) were diagnosed by liver biopsy.Their age of onset was 11,19 and 23 years old,respectively,with an average of 17.6 years old.The duration was 1-7 years.All the 3 patients were weak when they were young.The early symptoms of disease were anaemia,decreased blood platelet,hepatosplenomegaly,and portal hypertension or sinusoidal obstruction syndrome.Histologically,clusters of Gaucher cells with augmented volume were observed in expanded hepatic sinusoid of peripheral central veins and the cytoplasm of Gaucher cells were fibriform or crinkled.Immunohistochemistry revealed strongly positive expression of CD68 in liver tissue of the 3 cases,which was further demonstrated by double staining of CD68 and Hepa.No obvious diffuse hydropic degeneration of hepatocytes,focal inflammation or sinus inflammation was found.One case of Gaucher’s disease showed significant enlargement of portal area.The proliferative fibrous tissue was old and compact,which resulted in distortion of lobules and the formation of pseudolobule.Conclusions The 3 cases involved in present study show an earlier age of onset.They may have early changes of cirrhosis but the percentage of correct diagnosis is very low for the clinical characteristics lacked.Liver biopsy plays a very important role in the diagnosis of Gaucher’s disease.
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