[CIS-PAGID] questions about CGD

[YaeJean Kim]

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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