We report a case of two pseudolymphomas of the liver in a 63-year-old Japanese woman with primary biliary cirrhosis.One of the lesions was found incidentally during a medical examination,presenting as a 10 mm hypodens...We report a case of two pseudolymphomas of the liver in a 63-year-old Japanese woman with primary biliary cirrhosis.One of the lesions was found incidentally during a medical examination,presenting as a 10 mm hypodense nodule that revealed hyperdensity in the early phase and hypodensity in the late phase in computed tomography(CT)after injection of contrast medium.Retrospectively,the 10 mm nodule had first been discovered as a 4 mm nodule during CT 4 years previously.Superparamagnetic iron oxide-enhanced MRI revealed another 4 mm hyperintense nodule in segment 6 in addition to the 10 mm hyperintense nodule in segment 7.CT during arterial portography revealed two hypointense nodules.Findings with other imaging modalities such as ultrasonography,magnetic resonance imaging,and hepatic angiography were consistent with hepatocellular carcinoma.A right posterior segmentectomy was performed,and the lesions were microscopically diagnosed as pseudolymphoma.To the best of our knowledge,only 31 other cases of this disease have ever been reported,with a highly asymmetrical male:female ratio of 1:9.7.Although we could find only one case of transformation of hepaticpseudolymphoma into lymphoma in the liver,the exact nature of development from benign pseudolymphoma to malignant lymphoma is still not fully understood and cases of hepatic lymphoma need to be followed carefully.展开更多
Data from genome wide association studies and geoepidemiological studies established that a com-bination of genetic predisposition and environmental stimulation is required for the loss of tolerance in primary biliary...Data from genome wide association studies and geoepidemiological studies established that a com-bination of genetic predisposition and environmental stimulation is required for the loss of tolerance in primary biliary cholangitis(PBC).The serologic hallmark of PBC are the presence of high titer anti-mitochondrial autoantibodies(AMA)that recognize the lipoyl domain of the mitochondrial pyruvate dehydrogenase E2(PDC--E2)subunit.Extensive efforts have been directed to investigate the molecular basis of AMA.Recently,experimental data has pointed to the thesis that the breaking of tolerance to PDC--E2 is a pivotal event in the initial etiology of PBC,including environmental xenobiotics including those commonly found in cos-metics and food additives,suggesting that chemical modification of the PDC--E2 epitope may render its vulnerable to become a neo-antigen and trigger an immune response in genetically susceptible hosts.Here,we will discuss the natural history,genetics and immunobiology of PBC and structural constraints of PDC--E2 in AMA recognition which makes it vulnerable to chemical modification.展开更多
文摘We report a case of two pseudolymphomas of the liver in a 63-year-old Japanese woman with primary biliary cirrhosis.One of the lesions was found incidentally during a medical examination,presenting as a 10 mm hypodense nodule that revealed hyperdensity in the early phase and hypodensity in the late phase in computed tomography(CT)after injection of contrast medium.Retrospectively,the 10 mm nodule had first been discovered as a 4 mm nodule during CT 4 years previously.Superparamagnetic iron oxide-enhanced MRI revealed another 4 mm hyperintense nodule in segment 6 in addition to the 10 mm hyperintense nodule in segment 7.CT during arterial portography revealed two hypointense nodules.Findings with other imaging modalities such as ultrasonography,magnetic resonance imaging,and hepatic angiography were consistent with hepatocellular carcinoma.A right posterior segmentectomy was performed,and the lesions were microscopically diagnosed as pseudolymphoma.To the best of our knowledge,only 31 other cases of this disease have ever been reported,with a highly asymmetrical male:female ratio of 1:9.7.Although we could find only one case of transformation of hepaticpseudolymphoma into lymphoma in the liver,the exact nature of development from benign pseudolymphoma to malignant lymphoma is still not fully understood and cases of hepatic lymphoma need to be followed carefully.
基金Supported by National Institutes of Health grants (in part), DK39588, DK090019 and DK067003
文摘Data from genome wide association studies and geoepidemiological studies established that a com-bination of genetic predisposition and environmental stimulation is required for the loss of tolerance in primary biliary cholangitis(PBC).The serologic hallmark of PBC are the presence of high titer anti-mitochondrial autoantibodies(AMA)that recognize the lipoyl domain of the mitochondrial pyruvate dehydrogenase E2(PDC--E2)subunit.Extensive efforts have been directed to investigate the molecular basis of AMA.Recently,experimental data has pointed to the thesis that the breaking of tolerance to PDC--E2 is a pivotal event in the initial etiology of PBC,including environmental xenobiotics including those commonly found in cos-metics and food additives,suggesting that chemical modification of the PDC--E2 epitope may render its vulnerable to become a neo-antigen and trigger an immune response in genetically susceptible hosts.Here,we will discuss the natural history,genetics and immunobiology of PBC and structural constraints of PDC--E2 in AMA recognition which makes it vulnerable to chemical modification.
