[CIS-PAGID] non conditioned BMT in a SCIDs case

[Nelson, Robert P Jr]

Our health system, beginning with the pharmacy branch, is pondering the question of whether changing IVIG dosing guidelines from actual to ideal body weight would be low hanging fruit for reducing IVIG expenditures, without adversely affecting patient care outcomes.  Of course the initial question is general and not answerable across an array of immunodeficiency/autoimmune/neurological conditions.  But I thought this might be a good group to start with and ask if this strategy is being considered elsewhere or if you might have thoughts.....

You see, many Hoosiers are large and the savings would be significant.

Bob

Robert P. Nelson Jr., MD
Professor of Medicine and Pediatrics
Divisions of Hematology/Oncology
535 Barnhill Dr. Ste 473
Indianapolis, IN  46202
Telephone: 317-948-1186
E-mail: ronelson at iupui.edu<mailto:ronelson at iupui.edu>
pager:  317-312-1773

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Nelson, Robert P Jr
Sent: Friday, May 11, 2012 5:19 PM
To: 'pagid at list.clinimmsoc.org'
Subject: Re: [CIS-PAGID] non conditioned BMT in a SCIDs case

Dr. Mageed,

My opinion is that it is good enough, especially if the Hb and platelet counts are normal.

To me, the function is adequate to immunize to live viruses.

If the T cell numbers are stable rather than dropping, I would observe.   I don't think it is important whether the myeloid cells are donor or recipient.  What you gain with a boost is a minor lymphocyte nudge to 100%, if she is not there already, and risk graft-versus-host, which would subvert the goal of improving reconstitution.  I don't think that the mixed lymphocyte chimerism is the predominate immunophysiological influence on the stalled recovery, so that the boost may not even provide the hoped for result with respect to increased naïve T cell production.  The rather tepid production now may improve given more time and if the donor is a heterozygote for the unknown molecular defect, a boost would not be expected to help.

Where are you getting your TRECs measured, just curious, looking for a resource.

Bob



Robert P. Nelson Jr., MD
Professor of Medicine and Pediatrics
Divisions of Hematology/Oncology
535 Barnhill Dr. Ste 473
Indianapolis, IN  46202
Telephone: 317-948-1186
E-mail: ronelson at iupui.edu<mailto:ronelson at iupui.edu>
pager:  317-312-1773

From: pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org> [mailto:pagid-bounces at list.clinimmsoc.org]<mailto:[mailto:pagid-bounces at list.clinimmsoc.org]> On Behalf Of Aly.Mageed at helendevoschildrens.org<mailto:Aly.Mageed at helendevoschildrens.org>
Sent: Friday, May 11, 2012 1:15 PM
To: pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] non conditioned BMT in a SCIDs case

What does the group think of a 4YO with a SCIDs/Omenn's with an unidentified molecular basis who was transplanted from a full sib without conditioning more than 3 years ago? She is doing well clinically without serious/unusual infections. However, she has stalled in donor chimerism to only 10-14%, her lymphocytes are 96% donor (likely 100% if it were very pure without myeloid contamination). Yet, she continues with CD3/4/8 lymphopenia (CD3 is 400-1000), mostly 400-500. Most recent CD4 were 250. CD4RTE 27%, TREC is low at 280-350 (600-700/million CD3), CD19 last was 300. She is surprisingly maintaining her own IgG level 400-500. Despite the lymphopenia, PHA is good at 60-70% functionally. She responded well to non live vaccines with protective levels against Hep B, DPT and prevnar.
So the Qs are:

1.    Is this good enough at this stage since she is clinically well?

2.    Could she get live vaccines?

3.    Or, Does she need a graft boost?....
Thanks

Aly Mageed, MD, MBA
Pediatric Blood & Marrow Transplant
Helen DeVos Children's Hospital
aly.mageed at helendevoschildrens.org<mailto:aly.mageed at helendevoschildrens.org>




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