[CIS-PAGID] looking for transplant advice

[Sorensen, Ricardo]

Hi, I do not know if Matias Oleastro en Buenos Aires is reading this question (he will now), but I do know that he has dealt repeatedly with this situation in BCG-immunized SCID patients. I also do know that when the infection is disseminated, SCT does not work very well, but without it, its hopeless. In some SCID patients BCG actually does not disseminate and the local reaction appears after transplantation, but that is different and not bad. 

Ricardo Sorensen  

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Prof. Dr. Antonio Condino Neto
Sent: Thursday, June 23, 2011 2:53 PM
To: pagid at list.clinimmsoc.org; Sullivan, Kathleen
Subject: Re: [CIS-PAGID] looking for transplant advice

Dear Kate

All of my CGD cases that underwent BMT started receiving drugs for BCG at least 6 weeks before the BMT or even earlier if they developed complications with BCG (25% of them complicate with BCG). The drugs against BCG were given continuously, before and after the BMT procedure and ALL of them worsened during BMT procedure, bringing it to real risk that has to explained to the family. As the BMT engrafted the BCG was brought to control.

In your case I would try to have it as more stable as possible before BMT and do it as quick as possible, the patient´s only chance to cure it.

Hope this helps your decision

All best

Condino
--
Antonio Condino-Neto
Professor of Experimental Medicine
Institute of Biomedical Sciences, University of São Paulo 1730 Lineu Prestes Avenue, São Paulo - SP. ZIP 05508-000. Brazil Tel (55) (11) 3091-7387 / Fax (55) (11) 3091-7224



Citando "Sullivan, Kathleen" <sullivak at mail.med.upenn.edu>:


> 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

>

>

>




----------------------------------------------------------------
This message was sent using IMP, the Internet Messaging Program.