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Dear Colleagues
I would be grateful for thoughts on prophylaxis of SOS in patients with VLD. Patient in question has HVC BCS successfully treated by IVC venoplasty in the 90s with no current signs of portal hypertension or cirrhosis, although has not had a liver biopsy. Liver function normal. Has MPN which has progressed to MF and being worked up for bone marrow transplant. Haematologists understandably concerned about sinusoidal obstruction syndrome with busulfan conditioning.
I was thinking of liver biopsy to assess for cirrhosis. UDCA prophylaxis. Haematologists considering defibrotide but I appreciate the data for prophylaxis is scarce.
Many thanks for your consideration.
Kind regards
Dhiraj
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Dear 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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Hi 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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I 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 -
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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 -
Dear 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 -
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