[CIS-PAGID] questions about CGD

[Antonio Condino]

Prefer related donor

You have to stop IFNgama anyway 30 days befor transplantation

I do not think he developed autoimmune disorder

Antonio Condino

2012/1/9 YaeJean Kim <yaejeankim at skku.edu>


> Dear all,

>

> Greetings. Hope you all the heathly and happy new year ( this year is the

> year of Black Dragon!!)

> First of all, I would like to thank all the colleagues who responded to me

> early last year on a very sick baby with newly diagnosed CGD due to liver

> abscess. I managed to save him thanks to your advices and he is now 6 weeks

> out after successful stem cell transplantation and will go home this

> week!!  I really appreciate all the advices.

>

> Now, I have another question about the a new CGD patient (mom and sisters

> are carriers) who was referred for a stem cell transplantation last week

> and happened to have fever with oral ulcers on arrival (active ones in the

> throat, lips and healing one in buccal mucosa).  His parents wanted to get

> stem cells from one of his carrier sisters for him rather than from

> unrelated donors.

>

> This is a 6 year old boy who has been on itraconazole and interferon gamma

> at other instituion. Extensive infection history.. all managed to control

> and currently no active infection.

>

> He had  a mild chicken pox early last month. No other complication. Had

> another fever near Christmas which resolved after 3-4 days without

> complication. His fever this time resolved after 4 days and the oral ulcers

> improved. No other significant physical exam.

>

> Reviewing his lab, I found he had a quite high total protein (reaching

> 9) on chemistry and high ESR near 100, low CRP, no significant

> leukocytosis. I was wondering about any possibility of development of

> autoimmune disease after IFN-gamma treatment and sent out lab for

> autoimmune ds.

> Immunoglobulins are increased. No hx of IVIG tx. IgG 1,982 mg/dL, IgA 256,

> IgM 270. C3 and C4 are not decreased. FANA negative, anti-ds-DNA value

> increased.

>

> For the moment, I am planning to discontinue interferon and put him on

>  itraconazole and bactrim prophylaxis meanwhile. Talking to his mom, mom

> reported just feverish episodes for 3-4 days with ulcers or with

> generallized aches (?) without foci and fever used to disappear and she

> considered this was just flu or flu like sx related to interferon-gamma

> shots.

>

> My questions are below:

> 1. Any management issues for possible autoimmune ds due to

> interferon-gamma in CGD pts? esp. regarding before stem cell

> transplantation? I would stop this for the moment and see how he does.

> 2. Donor selection issue for transplant in terms of preferance: unrelated

> healthy donor vs. related CGD carrier donor vs. cord

>

> I would appreciate any advice or experience regarding these issues.

>

> Regards,

>

> YaeJean

> --

> Yae-Jean Kim, MD

> Assistant Professor

> Division of Infectious Diseases

> Department of Pediatrics

> Sungkyunkwan University School of Medicine

> Samsung Medical Center

> 50 Irwon-dong Gangnam-gu

> Seoul, Korea

> Tel)  +82-2-3410-0987  Fax) +82-2-3410-0043

> yaejeankim at skku.edu

>

>



-- 
Antonio Condino-Neto, MD, PhD
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
URL:
http://web.me.com/pereirapvs/Laborat%C3%B3rio_de_Imunologia_Humana/Home.html
Curriculum vitae: http://lattes.cnpq.br/5546433568874059
Pubications PubMed: http://www.ncbi.nlm.nih.gov/pubmed?term=condino-neto
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