[原发性血色素沉着症患者的肝脏病理学——C282Y突变的纯合子携带者]

[Liver pathology in patients with primary hemochromatosis--homozygous carriers of C282Y mutation ].

作者信息

Smirnov O A, Shneĭder O V, Radchenko V G, Nilova V K, Okon E E, Kudriavtsev B N, Saburova G S, Mitrofanov N A, Stanzhevskiĭ A A

机构信息

I. I. Mechnikov St-Petersburg State Medical Academy, 195067, St-Petersburg Institute of Cytology, 194064, St-Petersburg.

出版信息

Arkh Patol. 2003 Jul-Aug;65(4):51-5.

PMID:14518196

Abstract

Liver pathology was studied in 3 patients with primary chemochromatosis. In two cases so-called iron free foci with signs of hepatocytes with feature of dysplasia were found. Many siderosomes were found ultrastructurally in the cytoplasma of hepatocytes. Histological markers of virus infection were absent in a patient with positive serum HbsAg and HCV-Ab. Alcohol did not produce typical histological changes. In this case grave liver reticuloendothelial hemosiderosis typical for secondary hemochromatosis and overloading with iron of spleen pulp according to MR imaging were observed.

摘要

对3例原发性血色素沉着症患者的肝脏病理进行了研究。在两例中发现了所谓的无铁病灶，伴有具有发育异常特征的肝细胞迹象。在肝细胞胞质中通过超微结构发现了许多含铁小体。血清HbsAg和HCV-Ab阳性的一名患者未发现病毒感染的组织学标志物。酒精未产生典型的组织学变化。在该病例中，观察到了继发性血色素沉着症典型的严重肝脏网状内皮细胞含铁血黄素沉着症，并且根据磁共振成像显示脾髓铁过载。