[CIS-PAGID] looking for transplant advice

[Church, Joseph]

I would add the gamma interferon.  We had a non-adherent teen with HIV
and < 50 CD4+ cells for two years when he came in with disseminated MAI.
We gave him gamma interferon in addition to quad therapy.  He decided to
take his meds and a year later he had >200 CD4's and multiple negative
MAI PCRs.  He remains on his HIV meds and is off all AFB therapy.  The
gamma interferon was very well tolerated at "CGD doses."  JC

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Sullivan,
Kathleen
Sent: Thursday, June 23, 2011 12:20 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS-PAGID] looking for transplant advice

I am posting this question on behalf of others but the big question is:

Does it make more sense to transplant now or to try to achieve some
clearance of mycobacteria prior to BMT?

The patient is a two year old with very low T cell numbers (CD3 about
100-200) and no TRECS.  Her mitogens are not completely flat but about
2-4% of the control.  She has immunoglobulin and B cells and has had
some responses to vaccines.  She had PCP and now presents with MAI and
huge nodes.  She was treated with triple therapy for about three weeks
for her MAI and the nodes enlarged.  We have increased her MAI coverage
to 5 drugs and are thinking about adding gamma-interferon.  We do not
have a genetic type of SCID identified although she has a mutation of
uncertain significance in the IL-7Ra gene and she has uniparental
isodisomy of that chromosome.

Given this picture, what do other people think about hurrying to do a
transplant on the theory that this is the only curative maneuver that
can clear her MAI vs waiting to achieve some level of control and then
transplanting?


Kate

Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363





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