Venoocclusive disease in hepatic vein characterized by:
Correct Answer: Hepatomegaly
Description: Ans is b i.e. hepatomegaly Veno-occlusive disease (VOD) now better known as 'Sinusoidal obstruction syndrome' is one of the most frequently encountered serious complications after bone marrow transplantation. Other uncommon causes are: - Long-term immunosuppression with azathioprine in renal and liver transplant patients - Sporadic cases may be due to use of herbal teas (pyrrolizidine alkaloids). The hallmark features of VOD are hyperbilirubinetnia, painful hepatomegaly, and fluid retention. The onset of VOD usually occurs within 3 weeks after bone marrow transplantation, with a peak 12 days posttransplantation. However, the onset of VOD has been repoed even later. According to the modified Seattle criteria, 2 or more of the following must be present prior to 20 days after stem cell transplantation for a diagnosis of VOD: Bilirubin level of more than 2 mg/dL (34 prnol/L) Hepatomegaly and upper right quadrant pain of liver origin Ascites and/or unexplained weight gain of more than 2% above the reference range Pathophysiology: The causes of VOD are still unclear, but a combination of pretransplant risk factors and transplant-related conditions are believed to trigger a primarily hepatic sinusoidal injury. This can quickly extend to a hepatocytic and panvasculitic disease. This process eventually leads to complete venular obliteration, extensive hepatocellular necrosis, and widespread fibrous tissue replacement of normal liver. Because of the initiating pathology it is also called sinusoidal obstruction syndrome. The disease has significant morbidity and moality. Treatment is largely suppoive. VOD and Budd-Chiari syndrome In VOD hepatic venous outflow obstruction occurs from diffuse involvement of hepatic venules/ vein radicles, whereas in Budd-Chiari syndrome the outflow obstruction is due to involvement of major hepatic veins or the hepatic poion of IVC. In VOD the major hepatic veins are patent. Tender hepatomegaly with jaundice and ascites is common in both conditions.
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Surgery
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