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6 May 2021 at 16h24 #2752
Juan Carlos Garcia-Pagan
ParticipantDear Dhiraj,
I think that there is no data to support any of these possibilities. We have not personal experience in the issue. Sorry. Interesting to know and maybe collect potential cases.
Best
Juan Carlos -
6 May 2021 at 16h58 #2754
Andrea De Gottardi
ParticipantDear Dhiraj,
For the preparation of the VALDIG book that will include a chapter dedicated to this issue, Prof. Paul Richardson was rather supporting the use of DF in this indication. Prevention should be considered in particular in subjects at high risk with UDCA and DF, but data available refer to a limited numnber of patients and therefore need to be confirmed.
Kindest regards
Andrea
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7 May 2021 at 9h10 #2755
David Patch
ParticipantHi Dhiraj-wedge pressure at time of biopsy would be useful and if no portal hypertension then reassuring. Defibrotide has been used by haematologists for a long time but as others have commented, evidence is woeful. It does seem safe and well tolerated, and in the overall cost of a BMT is small beans!
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7 May 2021 at 9h21 #2757
Pierre-Emmanuel Rautou
ModeratorDear Dhiraj,
I completely share what has been said: scarce data but in this specific setting Defibrotide on top of UDCA seems reasonable.
Best wishes
Pierre-Emmanuel -
7 May 2021 at 10h29 #2760
Laure Elkrief
KeymasterDear all,
I fully agree.
Especially, I would perform a liver biopsy before BMT to assess for cirrhosis +++
best regards
Laure -
7 May 2021 at 10h38 #2761
Juan Carlos Garcia-Pagan
ParticipantI would like to add that the data on prophylactic fibrotide (quite expensive in Spain) is mainly in patients without severe choric liver disease. We are refering to a patients with BCS. A previous liver biopsy to stage the disease is very relevant, as well as other potential tools (HVPG, elastography, discarding presence of esophageal varices or oterh signs of PH) that can help to assess severity. In any case, if the hematologist finally accept to do the transplant (in our centers they usually are not prone to accept if we do not demonstarte that the liver problema is controlled and mild) I Will probably probably recommend some type of prophylaxis (although teh data is scarce and null in patients with previous liver disease).
Best -
7 May 2021 at 13h38 #2762
Tripathi Dhiraj
ParticipantDear Colleagues
Thank you very much for your helpful responses. I will do full liver assessment (LSM/histology/pressures). No varices. Agree data on prophylaxis is lacking but my liver assessment will guide me.
Regards
Dhiraj -
7 May 2021 at 14h23 #2763
Antonio Colecchia
ParticipantDear 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 -
3 August 2021 at 16h04 #2798
Tripathi Dhiraj
ParticipantDear Colleagues
Just as an update. HVPG 2 mmHG. Histology consistent with extramedullary haemopoiesis. No fibrosis, NRH or vascular/congestive features.You will recall history of BCS, and MPN which has progressed to MF and being worked up for bone marrow transplant. LFT’s normal.
Regardless, haematologist keen on some prophylaxis against SOS. Consider UDCA?
Regards
Dhiraj -
3 August 2021 at 22h38 #2799
Pierre-Emmanuel Rautou
ModeratorDear Diraj,
Thanks for the follow-up.
UDCA as been shown as beneficial as a prophylaxis in BMT (Ruutu Biol Blood Marrow Transplant. 2014), although the hepatic endpoint was rather vague. But it does not harm. In Paris, UDCA is very widely given as a prophylaxis in BMT.
Best regards
Pierre-Emmanuel
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