Clinical Diagnosis And Treatment Of Hepatic Sinusoidal Obstruction Syndrome

ObjectiveAnalysis the etiology, clinic characters, treatments and prognoses of 10 cases of hepatic sinusoidal obstruction syndrome (SOS). Meanwhile, summarize diagnosis techniques and common therapies of SOS at present.MethodRetrospective analyze 10 cases of SOS from 2010.09 to 2014.06 in Digestive Department, Provincial Hospital affiliated to Shandong University. Summarize the causes, clinical manifestations, complications, laboratory examinations and imaging examinations, liver biopsy pathologies, HVPG, treatments and prognoses, etc.Results1.10 cases came from Shandong Province. The men are more than women (male 8 cases, female 2 cases). Their ages vary from 25 to 76.2. In 10 cases of SOS,8 cases had histories of taking gynura segetum; 1 patient had a history of taking unknown composition Chinese medicine; the other 1 cases had no obvious causes.3. In 10 cases,5 patients had a history of hypertension,3 patients had a history of diabetes mellitus.4. Initial symptoms are fatigue, abdominal distension, edema of lower extremity, etc.5. The clinical manifestations include hepatomegaly in 7 cases, abdominal pain in 3 cases, ascites in 10 cases, jaundice in 5 cases, edema of lower extremity in 6 cases, and the weight gains in 1 case.6. Laboratory examinations indicated liver function injury:6 cases in Child-Pugh C degree,4 cases in Child-Pugh B degree. Increased CA125 of serum was observed in 5 cases.7. Imageological examination:The features of ultrasonic examination include hepatomegaly, ascites, hyperecho of liver parenchyma, thickness of the hepatic venous wall, and different degree stenosis of the hepatic venous lumen. Abdominal enhanced CT shows that:on plain CT scan, the liver density is uneven; on hepatic arteral phase scan, it presents grid-like enhancements; on portal venous phase, hepatic parenchyma demonstrate patchy and map—like changes, which distribute with low density and low perfusion area, no intrahepatic venule was noted. Portal venous collateral circulation can be seen in some cases.8.8 cases did electronic gastroscopy, and 4 cases presented mild to severe esophageal gastric varices.9.6 cases were taken liver biopsy. Pathological results showed that:hepatocytes are normal or slightly edema and cholestatic, hepatic sinusoid expand and congested, inflammatory cells infiltration in portal area.10.3 cases were taken bone marrow aspiration,2 of them indicated secondary polycythemia.11.5 cases were taken HVPG test:WHVP was 22.94±4.23 mmHg, FHVP was 2.92±2.50 mmHg, and HVPG was 20.02+5.00 mmHg, which indicates their HVPG are significantly elevated.12. Treatment and prognosis:All patients were given anti-coagulation, liver protection, diuresis, improving minicirculation and other treatments.3 cases took TIPS, and 1 of them cured,1 improved,1 died. In the other 7 cases,5 of them cured, and 2 died.ConclusionThe patient should be highly suspected of hepatic sinusoidal obstruction sy ndrome, if he/she has a history of taking gynura segetum or other traditional Chinese medicine (TCM), and has the following symptoms and signs:abdomin al distension, rapid growth of refractory ascites, edema of lower extremity, jau ndice, abdominal pain, etc.. In case of meet the Seattle or Baltimore prompt di agnosis standard, imageological examination would conduce to the diagnose. Hi storic confirmation with liver biopsy and HVPG>10 mmHg are regarded as gol d standards in diagnosis of SOS. SOS progresses fast, and it carry a high mor tality rate. Hence, early diagnosis and timely and appropriate treatment become s particularly important. Staging and typing is important in formulating effectiv e therapeutic planWhen comes to the treatment of SOS, the firt step is to remove predispos ing factors, such as hepatotoxicity drugs and high-intensity chemo-radiotherapy, or preventative applying of ursodeoxycholic acid two weeks before HSCT. Supp ortive treatment includes fluid management, adequate oxygenation and transfusio nal support to minimize ischemic liver injury, and avoidance of hepato/nephrot oxins. Switching over to drug therapy, in spite of liver-protecting drugs and diur etic drugs, DF demonstrates remarkable safety and tolerability, with manageable toxicities and low rates of attributable hemorrhage. The effect of low molecul ar weight heparin(LMWH) lacks authoritative account. Glucocorticoids may be considered for the use in the treatment of SOS with the appropriate caveats of caution regarding infection. When treat SOS with t-PA, life-threatening hemorr hages are common, and there’s no apparent survival advantage. Surgical therap y include transjugular intrahepatic portosystemic shunt (TIPS) and liver transpla ntation. TIPS relieve ascites in some patients with chronic SOS, but in some p atients with acute SOS, this procedure may lead to deteriorated condition and unimproved outcome. Liver transplantation, as the ultimate therapeutic tool for S OS, should be considered only in patients with severe liver failure who are ex pected to have a good outcome in the absence of liver disease, and those who have undergone bone marrow transplantation for benign disease.