[CIS PIDD] [cis-pidd] Sequential Lung and Bone Marrow Transplant

[CIS-PIDD]

Hi Alejandro,
This patient is 28 years old and has a syndrome similar to NEMO deficiency (ED + ID) but with a different genetic cause that we are investigating. Immunoglobulin levels are in the normal range but T, B, and NK cells are almost absent at this point. Historical lymphocyte phenotyping was never done, so I don't know if that was a lifelong problem. Not sure if yours is similar.
Best,
Jordan
National Jewish Health
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From: cis-pidd at lyris.dundee.net [cis-pidd at lyris.dundee.net] on behalf of CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Friday, June 09, 2017 2:12 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Sequential Lung and Bone Marrow Transplant

I do have a patient like that. 45 yo wm. No B cell, very low T cells, border IGG with low IgG2 and IgG4. Very severe bronchiectasis from early childhood. Is it similar to your case?

Alejandro Malbran
UAAIC
Buenos Aires


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________________________________
From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Thursday, June 8, 2017 2:44:17 PM
To: CIS-PIDD
Subject: [cis-pidd] Sequential Lung and Bone Marrow Transplant

Dear All,

I have an adult patient that needs both a lung transplant (severe bronchiectasis and cor pulmonale) and HSCT (novel syndrome with very severe lymphopenia). It looks like Children's Pittsburgh is doing the combination, and I am awaiting a response from that center. Does anyone else know of a center where this combined procedure is being performed in the United States? Anyone with a patient that has had both transplants performed? Any information is appreciated.

Thanks,
Jordan

Jordan Abbott, MD
National Jewish Health
Denver, CO, USA


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