Case 20 – summary

Veno-occlusive disease

Veno-occlusive disease is a post bone marrow transplant complication.

A combination of pre-transplant risk factors, and transplant related conditions are believed to trigger primary hepatic sinusoidal injury, although a definitive underlying cause is unknown.  This can lead to hepatocytic and panvasculitic disease, which is followed by multiorgan failure.

Histology –

  • Early –  widening of the subendothelial zone, red cell extravasation, fibrin deposition, and expression of factor VIII/von Willebrand factor within venule walls, followed by necrosis of the perivenular hepatocytes
  • Late –  deposits of extracellular matrix, an increased number of stellate cells, and subsequent sinusoidal fibrosis.
  • Overall this leads to complete venular obliteration and replacement of normal liver tissue with fibrous tissue.

Risk factors:

  • Preexisting liver disease
  • Second myeloablative hematopoietic stem cell transplantation (HSCT)
  • History of treatment with gemtuzumab ozogamicin
  • Allogeneic HSCT for leukemia beyond the second relapse
  • Conditioning with busulfan, melphalan, or both
  • Osteopetrosis
  • Macrophage-activating syndromes (eg, hemophagocytic lymphohistiocytosis, Griscelli syndrome)

Clinical symptoms:

  1. weight gain
  2. an increase in abdominal circumference
  3. hepatomegaly
  4. right upper quadrant pain
  5. ascites
  6. elevated total and direct bilirubin levels.
  7. transfusion-refractory thrombocyotopenia

VOD usually occurs within 20 days post transplant

Work up:

  • FBC
  • Chimerism
  • Bilirubin
  • LFTS
  • Coagulation, incl D-dimer
  • CRP
  • USS, DOPPLER. – diagnostic finding is reversal of flow in the portal and hepatic veins

Possible treatments:

Defibrotide –  a single-stranded polydeoxyribonucleotide derived from porcine tissue that possesses antithrombotic, thrombolytic, anti-inflammatory, and anti-ischemic properties

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