BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted b...BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells,which leads to progressive dysfunction of the affected organs.The two most commonly affected organs are the heart and kidneys,and liver is rarely the dominant affected organ with only 3.9%of cases,making them prone to misdia-gnosis and missed diagnosis.CASE SUMMARY A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly.Initially,based on enhanced computed tomography scan and angiography,Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed.However,addi-tional diagnostic procedures,including liver biopsy and bone marrow-exami-nation,revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression.The disease course was progre-ssive and fatal,and the patient eventually died 5 mo after initial presentation of symptoms.CONCLUSION AL amyloidosis with isolated liver involvement is very rare,and can be easily misdiagnosed as a vascular disease.展开更多
Congenital extrahepatic portosystemic shunt(CEPS),also known as Abernethy malformation,is a rare disorder characterized by the partial or complete diversion of portal blood into systemic veins.CEPS is classified into ...Congenital extrahepatic portosystemic shunt(CEPS),also known as Abernethy malformation,is a rare disorder characterized by the partial or complete diversion of portal blood into systemic veins.CEPS is classified into two types:typeⅠis an end-to-side portocaval shunt,whereas typeⅡis a side-to-side shunt,diverting blood from the portal vein to the inferior vena cava(IVC)(Figure 1).[1]展开更多
BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely reported.CASE SUMMARY Here,we describe the cas...BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely reported.CASE SUMMARY Here,we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer resection.Surprisingly,splenomegaly and thrombocytopenia were not accompanied by variceal bleeding,which has been reported to have predictive value for gastric variceal formation.However,a liver biopsy showed fibrosis in the portal area,suggesting NCPH.The patient underwent endoscopic treatment and experienced no further symptoms.CONCLUSION It is necessary to guard against long-term complications after oxaliplatin-based chemotherapy.Sometimes splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.展开更多
基金Tianjin Key Medical Discipline(Specialty)Construction Project,No.TJYXZDXK-034A.
文摘BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells,which leads to progressive dysfunction of the affected organs.The two most commonly affected organs are the heart and kidneys,and liver is rarely the dominant affected organ with only 3.9%of cases,making them prone to misdia-gnosis and missed diagnosis.CASE SUMMARY A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly.Initially,based on enhanced computed tomography scan and angiography,Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed.However,addi-tional diagnostic procedures,including liver biopsy and bone marrow-exami-nation,revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression.The disease course was progre-ssive and fatal,and the patient eventually died 5 mo after initial presentation of symptoms.CONCLUSION AL amyloidosis with isolated liver involvement is very rare,and can be easily misdiagnosed as a vascular disease.
基金funded by the Tianjin Health Technology Project(20188)Peking Union Medical Foundation-Ruiyi Emergency Medical Research Fund(R2020010)。
文摘Congenital extrahepatic portosystemic shunt(CEPS),also known as Abernethy malformation,is a rare disorder characterized by the partial or complete diversion of portal blood into systemic veins.CEPS is classified into two types:typeⅠis an end-to-side portocaval shunt,whereas typeⅡis a side-to-side shunt,diverting blood from the portal vein to the inferior vena cava(IVC)(Figure 1).[1]
基金Supported by Tianjin Science and Technology Plan Project,No.19ZXDBSY00030。
文摘BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely reported.CASE SUMMARY Here,we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer resection.Surprisingly,splenomegaly and thrombocytopenia were not accompanied by variceal bleeding,which has been reported to have predictive value for gastric variceal formation.However,a liver biopsy showed fibrosis in the portal area,suggesting NCPH.The patient underwent endoscopic treatment and experienced no further symptoms.CONCLUSION It is necessary to guard against long-term complications after oxaliplatin-based chemotherapy.Sometimes splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.
