Veno-Occlusive Disease (Sinusoidal Obstruction Syndrome)

  •         Pathology:
  • – Pathology mainly in central area
  • – Portal area is normal
  • – Destruction (non-occlusive) of sinusoidal and central vein endothelium
  • – Acute sinusoidal congestion and dilatation
  • – Acute sinusoidal obstruction cause abrupt ischemia as well as portal HTN
  •          Etiology:
  • – SOS is due to exclusively to agents that are toxic to both bone marrow progenitor cell and sinusoidal endothelium
  • Agents:
  •             – Irradiation of liver area
  •            – Chemotherapy
  •            – Immunosuppressive agents
  •            – Plant alkaloid
  • Common settings:
  •               – HCT
  •               – Chemo-radiation of abdominal malignancy
  •               – Immunosuppression with purine derivative
  •               – Oxaliplatin to metastatic CRC to liver

 

 

Figure: Sinusoid of Liver

 

  • Clinical features:
  • – Closely mimic BCS
  • – Asymptomatic
  • – Acute with ischemic necrosis of the liver
  • – Subacute/chronic with ascites
  • – Liver dysfunction of varying severity
  • Clinical diagnostic criteria in the setting of hematological stem cell transplantation (HCT):
  • – Weight gain/ascites
  • – Increased bilirubin
  • – Absence of other causes of liver dysfunction (sepsis, graft vs host disease)
  •               Diagnosis:
  • – Can be established only by liver biopsy after being confirmed patent large hepatic veins and IVC on imaging
  •                     Imaging:
  • – Liver is enlarged with a mosaic pattern suggestive of altered perfusion similar to BCS
  •                                                   Treatment:
  • – Treatment options have been mainly evaluated in the context of HCT
  • Prophylaxis:
  •                 • Decreased intensity of conditioning regimen
  •                • Defibrotide: anti-ischemic, anti-thrombotic and thrombolytic activity
  •                • Bevacizumab for cisplatin related SOS

 

 

Idiopathic Sinusoidal Dilatation

  • – Widening of hepatic sinusoidal lumen in the absence of post sinusoidal block/infiltration of sinusoid by abnormal cells or substance

 

Peliosis Hepatis

  • – Similar to idiopathic sinusoidal dilatation and is characterized by ‘Lake-forming’ hemorrhagic dilatation of sinusoids

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