Hepatic Sinusoidal Obstruction Syndrome:one Case Report With Literature Review

Objective:Hepatic sinus obstruction syndrome(HSOS),also known as hepatic veno occlusive disease(HVOD),is a relatively rare hepatic vascular disease.In recent years,the number of cases of pyrrolidine alkaloid-related hepatic sinusoidal obstruction syndrome(PA-HSOS)caused by taking plants containing pyrrolidine alkaloids(PAs)has been increasing year by year,and most of them have not been diagnosed and treated in time,resulting in poor prognosis.In order to further understand the hepatic sinus obstruction syndrome,we analyzed a case of hepatic sinus obstruction syndrome diagnosed in our hospital and reviewed the literature at home and abroad.By analyzing the etiology,clinical characteristics,diagnosis,differential diagnosis,treatment and curative effect,we can improve the understanding of the disease and reduce misdiagnosis and missed diagnosis.Materials and Methods:A case of hepatic sinus obstruction syndrome admitted to Union Medical Hospital affiliated Fujian Medical University in March 2018 was analyzed.Simultaneously,we searched and reviewed the literatures related with hepatic sinus obstruction syndrome in PubMed,China National Knowledge Infrastructure and Wanfang Data Knowledge Service Platform from February 1996 to February 2019,in order to analyse the the etiology,clinical manifestations,laboratory tests,imaging changes,liver biopsy pathology,treatment plan and treatment effect.Result:(1)General information:A total of 473 patients were included,among which 105were gender recorded in detail in case reports,59 were male(56%),46 were female(44%),104 were age recorded in detail,mainly middle-aged and elderly patients(74%).(2)Etiology:Among the patients in this group,348 cases of HSOS were reported in China,and 316(91%)patients had a history of taking Chinese herbal medicine.125cases of HSOS were reported abroad,111 cases(88.8%)had a history of HSCT.(3)Clinical characteristics:In this group,there were 105 cases with detailed records of symptoms and signs reported in individual cases,mainly manifested as abdominal distension in 96 cases(91%),ascites in 95 cases(90%),liver in 50 cases(48%),and jaundice in 46 cases(44%),accompanied by malaise,poor appetite,nausea,vomiting,oliguria,and edema of lower limbs.(4)Auxiliary examination:All the laboratory examinations collected in this group showed different degrees of hepatic dysfunction,mainly manifested by elevated transaminase and abnormal bilirubin metabolism,mainly with elevated DBIL.68patients underwent abdominal ultrasonography,mostly non-specific manifestations.In73 cases,abdominal CT examination showed hepatomegaly(26 cases,36%)and inhomogeneous decrease of liver density(40 cases,55%)during plain scan,heterogeneous enhancement of liver parenchymal density(39 cases,53%)during enhancement,and some patients also showed hepatic vein narrowing,fine or non-imaging changes(34 cases,47%).MRI of the upper abdomen also showed similar findings to those seen in abdominal CT.Among them,13 cases were diagnosed as hepatic sinus obstruction syndrome or hepatic venule occlusion by CT/MRI.Among them,13 cases were diagnosed as HSOS or HVOD by CT/MRI.Liver puncture was performed in 54 patients.The pathological results showed congestion and dilatation of hepatic sinuses,accompanied by swelling,degeneration,liquefaction necrosis of hepatocytes and infiltration of lymphocyte-dominated inflammatory cells.Some patients may have central vein or sublobular vein endometrial thickening and stenosis.(5)Diagnosis and misdiagnosis:Among the 105 cases,51 cases were clinically diagnosed and 54 cases were pathologically diagnosed.The average time from onset to definite diagnosis was 1.54±1.97 months.22 cases were misdiagnosed,and the misdiagnosis rate was 21%.Among them,11 cases were misdiagnosed as Budd-Chiari syndrome.(6)Treatment and curative effect:Among 318 PA-HSOS patients included in this group,97 patients(group A)received basic supportive treatment such as liver protection,diuresis and/or improvement of circulation,the improvement or cure rate was 45%;126patients(group B)were treated with anticoagulation,the improvement or cure rate was67%;29 patients(group C)were treated with glucocorticoid,the improvement or cure rate was 79%;51 patients(group D)were treated with anticoagulation combined with glucocorticoid stimulation.The improvement or cure rate of vegetarian therapy was69%.Compared with group A,group B(?~2=10.977,P=0.001),group C(?~2=10.334,P=0.001)and group D(?~2=7.271,P=0.007)showed statistically significant differences,with better efficacy than group A.There was no significant difference between group B and group C(?~2=1.567,P=0.211),the curative effect of the two groups was similar.Compared with B(?~2=0.023,P=0.88)and C(?~2=1.058,P=0.304),there was no significant difference in the curative effect of group D.The curative effect of group D was not better than that of group B and C.Among those ineffective in medical treatment,11cases were treated with TIPS.The improvement or cure rate was 82%.Among them,1 case was improved after surgical shunt operation.Liver transplantation was performed in 3 cases and improved in 2 cases.Conclusion:(1)Chinese herbal medicine containing PAs is the main cause of HSOS in China,while hematopoietic stem cell transplantation,organ transplantation and high dose radiotherapy and chemotherapy are the main causes in foreign countries.(2)HSOS should be vigilant in patients with unexplained ascites,hepatomegaly and jaundice.For patients initially diagnosed as BCS,attention should be paid to excluding PA-HSOS.(3)Anticoagulant drugs should be used as early as possible for PA-HSOS.The results showed that the efficacy of glucocorticoid therapy was similar to that of anticoagulant therapy,and the efficacy of glucocorticoid combined with anticoagulant therapy was not better than that of anticoagulant or glucocorticoid alone.