[CIS PIDD] [cis-pidd] lung transplant in CVID patients with GLID?

Fri Jul 17 02:08:00 EDT 2015

Dear Charlotte,

yes, I agree, we also had one CVID patient with lung transplant, I believe
we have already shared some data. I paste here a short history provided by
my colleague. Our patient died 24 months after Tx.
We would be happy to share our patient (unfortunatelly our records on the
critical period after transplantation show some gaps as we do not have full
access to data from transplantologists).

Regards

Anna Sediva

Our patient with Common Variable Immunodeficiency underwent lung
transplantation 20 years after diagnosis of CVID. Since his childhood he
suffered from recurent upper respiratory (incl. sinusitis, tonsilitis,
otitis media) and lower respiratory tract infections (incl. bronchitis)
complicated by bronchiectasis and chronic obstructive bronchopulmonary
disease. Because of severe progression he underwent bilateral lung
transplant in 12/2004. Post- transplant period with necessary combined
immunosupression (tacrolimus, mycophenolat mophetil, prednison),
antibacterial (cotrimoxasol) and antifungal prophylaxis (fluconazol) was
without serious complications and with very satisfying quality of life till
9/2006. Since that he suffered from recurrent lower respiratory tract
infections, which led to failure of donor lungs. Our patient died 12/2006,
24 months after transplant.

On Thu, Jul 16, 2015 at 8:52 PM, Cunningham-Rundles, Charlotte <
charlotte.cunningham-rundles at mssm.edu> wrote:

> We have done 3 lung transplants; they lived for 6  years ( 2 of them) and
> a few days post in the third.  The ones who did ok had thrombocytopenia,
> platelet dysfunction and died of that with lung bleeds.
>
>
> One other  in Canada I know of, (pt I saw before) is now 1 year out, lung
> and liver; has thrombocytopenia and neutropenia.
>
> I’d suggest that you use any medication regimen to put off transplant if
> you can. Transplant sets the clock on a count down.
>
>
>
> Between our group here, it would be really great to collect all our CVID
> transplant experiences.
>
>
>
>
>
> Charlotte Cunningham-Rundles, MD, PhD
> Departments of Medicine and Pediatrics
> The David S Gottesman Professor
> The Immunology Institute
> Mount Sinai School of Medicine
> 1425 Madison Avenue
> New York, NY 10029
> Phone: 212 659 9268
> Fax: 212 987 5593
> Email: Charlotte.Cunningham-Rundles at mssm.edu
>
>
>
>
>
>
> On 7/16/15, 12:35 PM, "Elena Hsieh" <whsieh at stanford.edu> wrote:
>
> >Thanks Dr. Routes.  Our patient received four courses of rituximab, and
> >about 6-8 months of imuran; she was getting better then recently
> >deteriorated and developed pulmonary hypertension.  I have indeed found
> >reports of lung transplant for bronchiectasis in CVID, the issue is
> >whether that experience could be extrapolated to GLILD.
> >
> >Thanks for your thoughts.
> >
> >Elena
> >
> >> On Jul 16, 2015, at 07:52, Routes, John <jroutes at mcw.edu> wrote:
> >>
> >> HI Elena
> >> I am not aware of any studies on this, although my guess is there are
> >> isolated case reports‹there are reports on lung transplantion in CVID
> >>and
> >> bronchiectasis with or without hypogammaglobulinemia‹
> >> you didnt mention how many courses of RTX the patient got and how long
> >>Rx
> >> with immuran‹‹we have retreated some or our patients
> >> also, might want to consider pulse methylprednisolone and see if she has
> >> any reversible component
> >> good luck
> >> Jack
> >>
> >> John M. Routes, MD
> >> Chief, Section of Allergy and Clinical Immunology
> >> Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
> >> Department of Pediatrics
> >> Children's Hospital of Wisconsin
> >> Medical College of Wisconsin
> >> 9000 W. Wisconsin Ave.
> >> Milwaukee, WI  53226-4874
> >> Phone: Office 414-266-6840
> >> Fax: 414-266-6437
> >> Email: jroutes at mcw.edu
> >>
> >>
> >>
> >>
> >> On 7/16/15, 12:02 AM, "Elena Hsieh" <whsieh at stanford.edu> wrote:
> >>
> >>> Hi there,
> >>>
> >>> We have a very sick 48yo woman with CVID and granulomatous-lymphocytic
> >>> interstitial lung disease (GLILD), who has now developed pulmonary
> >>> hypertension, s/p rituximab and imuran, currently not doing well.  Is
> >>> there a precedent for lung transplant in CVID patients with GLILD who
> >>> fail improvement with immunosuppressive regimen? If so, could you point
> >>> me to a good reference?
> >>>
> >>> Thanks.
> >>> Elena
> >>>
> >>> Instructor
> >>> Allergy and Immunology
> >>> Stanford University
> >>>
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-- 
Prof. Anna Sediva, M.D., Ph.D.
Department of Immunology
2nd School of Medicine, Charles University
University Hospital Motol
V Uvalu 84
Prague, 15006
Czech Republic

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