[CIS PIDD] [cis-pidd] AW: Low T-cells post-Heart Transplant

Wed Apr 26 00:29:21 EDT 2017

Hi Joe!

The best estimate for the chances of post-Fontan (w/o post-HTx medication) T cell count recovery is probably given in these articles:

J Clin Invest. 2016 Mar 1;126(3):1126-36. doi: 10.1172/JCI84997.

J Allergy Clin Immunol Pract. 2016 May-Jun;4(3):491-6. doi: 10.1016/j.jaip.2015.11.034.

Interindividual variation is rather large it seems...

So chances - with all that he has undergone - might be rather bleak? But of course, only an educated guess ...

How do you plan to approach his treatment/follow up??

Mikko

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)
[X]

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 26.4.2017 kello 6.39:

Dear Joe Church,

unfortunately especially in the setting of heart transplantation frequently almost total thymectomy is done. Once the patient is off prednisone, you could check recent thymic emigrant counts and naive/memory T cell count. I personally would start TMP/SMX prophylaxis and keep it if necessary lifelong (as opposed to the guidelines of cardiologists at least in Germany) as we had some bad experience with PCJ infection in this patient group.

Best, Fabian Hauck

Dr. von Hauner Children's Hospital Munich

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Von: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>> im Auftrag von CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Gesendet: Mittwoch, 26. April 2017 02:29
An: CIS-PIDD
Betreff: [cis-pidd] Low T-cells post-Heart Transplant

Colleagues:

I follow a 23yo young man who was born with hypoplastic left heart.  He had the usual surgeries including a Fontan procedure.  He developed very severe protein-losing enteropathy (albumin levels ~2mg/dL).  By 17yo he had pan-hypogammaglobulinemia and T-lymphopenia (CD4 worse than CD8).  Despite the numbers he really did not experience severe, chronic or opportunistic infections.

He received a heart transplant in June 2016.

As expected his serum albumin has gradually improved – latest 3.6.  However, he still has profound pan-lymphocyte deficiency:  CD3+ 46% (97), CD4+ 9% (18), CD8+ 34% (70).

He is still on prednisone (5mg/day), tacrolimus and mycophenolate.

What is the likelihood that he will repopulate his lymphoid populations?

Joe Church
Children’s Hospital Los Angeles

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