[CIS-PAGID] questions about CGD

[Jason W. Caldwell]

I can't comment on the donor issue, but when reading your case, I kept thinking of inflammatory bowel disease.  The ulcers are in his mouth, but I think I would check his gut for lesions and granulomas.  I think the histology here would be helpful and steroids for inflammation in this disease is usually very effective as long as he is infection free and on his prophylaxis.

Jason Caldwell
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of YaeJean Kim
Sent: Monday, January 09, 2012 4:23 PM
To: pagid
Subject: [CIS-PAGID] questions about CGD

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<mailto:yaejeankim at skku.edu>

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