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24 November 2020 at 9h00 #2630
Dear Valdig colleagues
we have been asked to perform TIPS in 4 patients with common variable immunodeficiency who have NRH on biopsy (as opposed to granulomatous type disease). In two patients there is significant ascites, and in the other two there is documented portal hypertension and elevated HVPG. Can I ask the forum what is your collective experience in this group of patients? It may be worth pooling this experience as the literature is minimal.
24 November 2020 at 11h30 #2632Antonio ColecchiaParticipant
The question is TIPS before Hematological stem cell transplantation (HSCT)
I am following many hematological patients before and after allogenic HSCT, being a coordinator of an italian multicenter study ( ELASTOVOD study ) on the use of liver stiffness in prediciting VOD after HSCT; in about 800 patients I have never seen perform TIPS before transplantation and in reality few italian centers insert TIPS also after HSCT to treat VOD ( even if in a recent paper by Ignacio Gomez-Centurion BBMT 2020, it seems be useful). However in your specific case ( TIPS before HSCT) considering the presence of PH and the high risk to develop VOD and other complication, I believe that to perform TIPS to reduce PH it might be an idea to consider ( no specific study have beeen carried out on this topic) and furthermore in the case of HSCT I could suggest to use also defibrotide in prophylaxis to prevent VOD
All the best
Antonio Colecchia MD
Borgo Trento University-Hospital
24 November 2020 at 17h36 #2633Juan Carlos Garcia-PaganParticipant
Dear Friends, As some of you already now we are collecting a large multicenter cohort of patients with IPH whithin the VALDIG network. In this cohort (more than 600 patients there are 5 patients with ICV that received a TIPS. All of them for refractory ascites. All of them died without the possibility to be transplanted because of severe comorbidities (one of these patients is from our center). Approximately one year ago we were requested to do a TIPS in a young female with severe portal hypertension that required a bone marrow transplantation. TIPS procedure was very well tolerated and bone marrow succesfully transplanted. She is still on flllow up.
I hope this may be of help
Juan Carlos Garcia-Pagán
Barcelona Hepatic Hemodynamic lab
27 November 2020 at 16h09 #2635
thank you both-clearly this is a niche group of patients.
I am happy to any database and I am hoping to get someone on board who can do the hard miles!
I will feed back your experience-I think the push will be towards TIPS -and I can then feed back the outcomes
24 March 2021 at 14h47 #2721
I thought an update would be useful re this patient
TIPS went ahead with control of ascites, and following initiation of avapritinib, he is back at work and no ascites etc.
So based on the experience of one(!) we have been referred another…
24 March 2021 at 22h17 #2723Dominik BettingerParticipant
indeed, it is an interesting topic performing TIPS implantation in patients with common variable immunodeficiency. Recently, we published a small case series of TIPS implantation in these patients (J Allergy Clin Immunol Pract. 2021 Mar 12;S2213-2198(21)00301-9). In line with the results mentioned by Juan Carlos Garcia-Pagán, the outcome was not very good, especially as some patients developed infectious complications. Hope this may help.
University Medical Center Freiburg, Germany
25 March 2021 at 5h50 #2725Stephen CaldwellParticipant
Appreciated these notes. I’m still finding my way through Valdig but I am finding these exchanges to be really interesting. Indeed liver disease in CVID seems to be a niche condition. I have only several of these cases but haven’t polled colleagues to see what our total might be but I suspect very small. I’ve encountered both NRH and granulomatous injury with some success in one patient with colchicine/urso combined with spreading out doses of IVIG in controlling inflammation and ascites. Do any of you have an idea of the mechanism at work? I could not pull up Dominik’s article – please check the reference. I will soon see a now retired colleague with CVID and progressive very cholestatic enzyme pattern with new onset ascites in past 3 months – relatively young woman. Thanks, Steve
25 March 2021 at 14h20 #2726Dominik BettingerParticipant
I copy the citation and the PIMD in the message:
Globig AM, Heeg M, Larsen CS, Ferreira RD, Kindle G, Goldacker S, Strohmeier V, Silva SL, Cunningham-Rundles C, Quinti I, Thimme R, Bettinger D, Schultheiß M, Warnatz K. International multi-center experience of transjugular intrahepatic portosystemic shunt implantation in patients with common variable immunodeficiency. J Allergy Clin Immunol Pract. 2021 Mar 12:S2213-2198(21)00301-9. doi: 10.1016/j.jaip.2021.02.056. Epub ahead of print. PMID: 33722695.
I hope you can receive this short communication, unless I can also send you the unedited print.
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