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

Thu Jul 16 14:52:43 EDT 2015

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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