Dear Dhiraj
Your case is very interesting and I agree with the others who are saying that experiences are scanty; I agree with Patch in considering staging BCS because in case of PH and high fibrosis degree the patient would be at high risk of developing VOD.
I might agree in performing invasive methods to stage the liver disease (biopsy and HVPG) even if ultrasound and liver and spleen elastography could be useful with almost the same effectiveness. In the setting of Stem cell transplantation (SCT) there are now some small studies in which liver stiffness was used to predict VOD development; Our group found (performing stiffness measurement before and after SCT once a week) that an increase of the stiffness after SCT can allow pre-clinical diagnosis of VOD (Colecchia BBMT 2019); and Karlas (BBMT 2019) found that baseline Liver stiffness can predict VOD development
Evidence of Defibrotide in prophylaxis in adult does not seem as good as in paediatric population (international trial in adult is ongoing), however it does seem safe and well tolerated
I believe that Defibrotide could be administered according to the stage of the disease following this strategy: a) prophylactic way in the case of high grade of liver disease assessed at baseline by elastography or as you want ( biopsy and HVPG) or b) pre-emptive way (in the case of increase of Liver stiffnes after SCT) if the liver disease is at low risk at baseline
Regards
Antonio
