Siliang Wu

1Affiliated Hospital of Hunan College of Traditional Chinese Medicine,Zhuzhou,412000.

Introduction

Hepatic veno-occlusive disease (HVOD),also namedsinusoidal obstructionsyndrome.There was avery detailed descriptionofHVODby Hilletc in 1953[1]. The main symptoms are painful hepatomegaly、fluidretention and jaundice.The treatmentof this diseaseisnot mature,symptomatic relief and supportivetreatmentisthe basic measure.The mortality rateof HVODis almost40%,so the prognosisevaluation mattersalot.Nowadays HVOD ismainly causedbySedumaizoon in China,and its prognosis dependents on thedosage and courses oftheSedum aizoontreatment.This case wasfound a obviousincreasedCA125level inthe serum,hydrothorax and ascites by accident,followed by thelivercirrhosiswithin3 months and then died.By nowwe guess that CA125levelcould forecast thelivercirrhosis followingbyhepatic veno-occlusive.Following is the case report.

Case report

The Patientwas a67 years’old woman,hospitalized fortheabdominaldistention and both lower extremitiesin January 2016.The patient toldusthat shehadthe signsof abdominal distension withno reason1monthago,and thenappearedbothlower extremities,nausea,poorappetite and right upper abdomen distending pain atthe same time.Abdominalultrasonography showedenlargement of theliverparenchymaspot,hepaticcyst,gallbladder pathologic changes,common bileductupside expands,pelvic and peritonealeffusions.Shehad a medicalhistory of Coronaryheart disease,and was hospitalized as “Edema causing:rightheart failure?”.Bodycheck showedthatBP 130/70mmhg,bothlungbreath soundsclear,bothinferior lung breathsounds slightlylow,noobvious dry and moistrale,HR74 timesper minutes,noarrhythmia,,no big murmur.Shehada grossly distended abdomen,butnot extendedto theliver and spleen,but shifting dullness positive.Theliver function results:AlanineTransaminase54u/L,glutamic oxalacetic transaminase 60u/L,Total protein 24.9g/L, total bilirubin 25.36umol/L, direct bilirubin 15.26umol/L.Urinarysediment showed thatthe urineproteinwasnegative.Epigastrium CT resultsshowed:liver congestion,Inferiorvena cava Liversegment stenosis,Hepatic vein notclear display,mass peritoneum effusion,retroperitoneal effusion.All theseabove indicated the possibility of Budd-Chiarisyndrome.ButWhen asked,the patientsaid thatshegrowSedum uizoonherself and then dried and grindedSedumuizooninto powder tobe takenafter beinginfused in boiling water,one spoonful,a time a day,had already lasted for 3 months.She taken the law material with her,and those were Chinese Herbs named Gynura japonica(known as Sedum uizoon)verified by Department of Pharmacy of Hunan Traditional Chinese Medical College.By now we considered her illness as HVOD but not Budd-Chiari syndrome,which need MRI to verify.Epigastrium MRI results showed:liver congestion,Inferior vena cava Liver segment stenosis,Hepatic vein definite stenosis,mass peritoneum effusion,retroperitoneal effusion.For the patient denied liver biopsy，she was treated by Albumin supplementation， diuresis and liver protection therapy.After the treating,the patient appeared double lower limbs edema abates,relieved abdominal distention and promoted appetite,so she left hospital 20 days later.

The patient was hospitalized again in February 2016 for the exacerbation of abdominal distention and both lower extremities for 7 days.Abdominal ultrasonography results showed:intrauterine solid nodule,undetermined properties.The gynaecologist suggested that we shall check the CA125 levels.The results were:serum CA125 > 1000u/ml,Hydrothorax and Ascites CA125>1000u/ml.The thinprep cytology test didn’t detect any malignant cell,and the CT Scan+enhancements of the Chest and Whole Abdomen showed no space occupying lesion. So she was treated by Albumin supplementation， diuresis and liver protection therapy,Jaundice-abating and pleural effusion and ascites percutaneous catheter drainage therapy.The patient left after remission.

The patientwas hospitalized again inApril2016 for theexacerbationofabdominaldistentionand bothlower extremities for2days.The patient was diagnosedas HVOD,decompensatedliver cirrhosis,andappeared recurrent episodesofgastrointestinal hemorrhage after 1 week,thenwastransferred to ICU,with nocontrolledbleeding,thendiedafter do-not-resuscitate determined by her family member.

Discussion

Although there is only one words’difference between Tu sanqi and sanqi,they are completely different drugs.Tu sanqi,also known as Sedum uizoon and Gynura segetum, belongs to Crassulacaeae.The stem is cylindrical,and the leaves are oblanceolate when flattened.The flower is yellow,the root is round and loose.While panax notoginseng belongs to the family pentagaceae,with upright stems,palmately compound leaves,green and umbrella-like flowers,round roots and solid texture.Due to the similar functions of promoting blood circulation,removing blood stasis,removing swelling and pain,and their names are similar,it is easy for patients to take the wrong medicine.Due to the presence of pyrrolidine alkaloids in Sedum uizoon,it may cause hepatic venous occlusion syndrome once mistaken Sedum uizoon,which is the main cause of hepatic venous occlusion syndrome in China.

Since the 1980s,when hou jingyan first reported that taking Sedum uizoon resulted in hepatic small vein occlusion syndrome,there had reports on and off.However,the current research mainly focuses on two aspects.First,the clinical characteristics of the disease,Peng Miao Miao had recruited 59 patients and Hao Jungui had recruited 25 patients for study,to explore the gender,age,dosage,time,primary disease,success rate of treatment and other factors in this patient[3-5].Second,the diagnostic value of imaging in the disease,their emphasis is on that abdominal CT and MRI have important diagnostic value in this disease[6-7].At present,there are few studies on the role of serology in this disease,especially serological indicators have not been found as an assessment of the prognosis of the disease.

Wedidn’t doliverbiopsy,butthe patienthad a history ofSedumaizoontreatment,andalsohadthe painful hepatomegaly、fluid retentionand jaundice symptoms,besides,liverCT and MRIboth prompted the hepaticvein stenosisandocclusion,so this casewasdiagnosedashepatic venoocclusivedisease.There is solidnodule inthe pelvis after the B-ultrasound examination,we excluded the malignancy possibility by CT scanningthechestand the whole abdomen and enhance,and thinprepcytologictesting,but we foundby accident that CA125 level obviously increasedin theserum、hydrothorax and ascites,so we considered that increased CA125 level correlated with HVOD.

CA125is ahigh molecularweightglycoprotein foundon the surface ofEmbryonic epithelialcells,now wildly usedasgynecological tumor marker,which correlates deeplywithliver cirrhosis[3-4].Therearetwo mechanisms lead toincreasedserum CA125levels followingliver cirrhosis[5].The peritonealmesothelial cellshaveahigher CA125 expressionduringtheirproliferation stimulatedby peritonealpressure rising,result inlarge plenty of CA125secreted into theabdomen.Besides,the serum CA125 levels increased is also causedbythe liverclearance abilityweakendueto theliver tissue structure damagedandmicrocirculationdisturbance.Sedum aizoon willcauseliversmallvenous endothelialcellsdamage,edema and blocking,liver tissue structure damaged and microcirculation disturbanceby lipid peroxidation orinducing apoptosisandsoon.Thispatient,however,was diagnosedmassascitesgivingriseto peritoneal pressureincreasedseveral times，followedby the high CA125 levels.So we assume there is positive correlation between the increased CA125 levels and the liver tissue structure damaged and microcirculation disturbance.

This patient was hospitalized three times in our hospital,and the first time had not yet developed into liver cirrhosis,but was found an increased CA125 levels at the second time,then died in the liver cirrhosis decompensatory period,her total process was just 3 months.So we guess that the obvious increased CA125 levels could indicate poor prognosis of the HVOD.This time we didn’t observe dynamic variation of the CA125 levels，neither did the biopsy of the pelvic nodal,besides,with no more cases,the reliability may decline.But nowadays the CA125 levels had not yet been reported correlating with HVOD,we hope this paper could evoke clinicians’attention to the CA125 levels of the HVOD patients,and adding more case reports to further verify the relationship between the CA125 levels and HVOD.

1. Hill K,RhodesK,Stafford J,et al.Serous hepatosis:a pathogenesis of Hepatic fibrosis in Jamaican children.Br MedJ1953,1:117-122.

2. Senzolo M,GermaniG,Cholongitas E,et al.Venoocclusivedisease:update on clinical management.World JGastroenterol 2007,13:3918-3924.

3. PengMM,GeWH,ZhugeYZ.Analysis of clinical featuresof patientswithhepatic venous occlusioncausedbysoilnotoginseng.Chin J Clin Pharmacol2018,34:938-941.

4. Hao JG,Yan XB,Ji F.Analysisof clinical features of 25 cases of hepatic venous occlusion causedbysoilnotoginseng.Liver 2017,22:108-110.

5. Li WH,WangXL.The diagnostic value of abdominalmriinpatients with liver damage caused by oral tupanax notoginseng.Management of healthstandardsinChina2018,9:119-121.

6. ShiYB.CT andMRIfindings of hepatic venous occlusion induced by tuanqi.Journal of ChengdeMedical College2018,35:210-211.

7. FuWY,ZhuWH.Acaseof hepatic venous occlusioncaused bysoil notoginsengwas diagnosed bythree-stagescanof64 rows of MSCTliver andCTV imaging.Functionaland molecularmedicalimaging 2018,7:1397-1399.

8. Qureshi MO,DarFS,Khokhar N.Cancer antigen-125 asamarkerof ascites inpatients withlivecirrhosis.Journalof the college of Physician andSurgeons-Pakistan:JCPSP 2014,24:232.

9. Le P C Y,Welten A G A,Ter W P M,et al.A peritoneal dialysis regimen low in glucose and glucose degradation products results in increased cancer antigen 125 and peritoneal activation .PeritonealDialysis International 2012,32:305.

10.Kalantri Y,Naik G,Joshi SP,et al.Role of cancer antigen-125 from pleural ascitic fluid samples in non malignant conditions.Indian J Med Res JT 2007,125:25.