[CIS-PAGID] questions about CGD
Prof. Dr. Antonio Condino Neto
condino at icb.usp.br
Tue Jan 10 06:43:23 EST 2012
Prefer related donor
You have to stop Interferon gamma anyway before BMT
--
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 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
>
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