8 例肝淀粉样变性的临床病理分析.

Objective To investigate the clinicopathological features of hepatic amyloidosis. Methods A retrospective analysis was performed for 8 patients with hepatic amyloidosis confirmed by liver biopsy in 302 Hospital of PLA from January 2005 to December 2012. Clinical data, laboratory examination results, imaging findings, and pathological data were analyzed to summarize the clinicopathological features of hepatic amyloidosis. Results The clinical manifestations and signs of this disease included hepatomegaly (7/8), weakness (6/8), poor appetite (6/8), splenomegaly (4/8), ascites (2/8), portal hypertension (2/8), edema of lower extremities (2/8), pruritus (2/8), a history of heart disease (2/8), and varices (1/8). Laboratory examination showed increases in alkaline phosphatase (ALP) (8/8) and gamma-glutamyl transpeptidase (5/8), hypoproteinemia (2/8), abnormal alanine aminotransferase (2/8), a slight increase in aspartate aminotransferase (1/8), and abnormal total bilirubin (1/8). As for imaging examinations, ultrasound showed uneven echo of liver parenchyma (8/8); transient elastography showed a significant increase in liver stiffness (2/2); CT showed diffuse weakness (4/8) or focal weakness (2/8). As for histopathological and immunopathological features, HE staining showed the deposition of pink substances around the hepatic sinusoids (8/8) and the central vein (6/8), Congo red staining of the pink substances obtained a positive result (8/8), and the pink substances showed a color of apple green under a polarization microscope; therefore, the patients were diagnosed with amyloidosis. As for immunohistochemistry, of all patients, 6 had positive Kappa staining and 4 had positive Lamda staining. Conclusion Hepatomegaly and elevated ALP are relatively common in patients with primary hepatic amyloidosis. When a patient has hepatomegaly and elevated ALP and cannot be diagnosed with common liver diseases, clinical physicians should consider the possibility of hepatic amyloidosis, which is rare but fatal, and make a confirmed diagnosis based on related examinations, in order to provide guidance for clinical treatment. [ABSTRACT FROM AUTHOR]