[PAGID] Disseminated mycobacterium

[Salem Tamemi]

I had one patient with complete INFGR II deficiency. She was on
clarithromycin and ciprofloxacin prophylaxis after being treated for
disseminated BCG vaccine infection. She was put on INF-g therapy but I
didn't think it helped her as much as the prophylactic antimicrobials. In
complete INFGR deficiency the serum INF-g level is very high already so one
would wonder how is it going to help, unlike in partial deficiency where it
may help. Clinically was failing to thrive and had significant
lymphproliferation including significant hepatosplenomegaly (I was not quite
sure the lymphoproliferation was because of the disease it itself or
secondary to the INF-g) anyway for the 1st time we transplanted her from a
matched sibling donor and remarkably done quite well, now 1.5 years post
transplant free from infections with complete resolution of
lymphoproliferation and she is growing. 

To note also, I follow patients with CGD who presented initially with
pulmonary mycobacterium infection but not aggressive and disseminated as
this patient which is usually the case in complete INFGRD, still worth
looking for it in this patient.

 

Salem Al-Tamemi, MD,FAAP,FRCPC.

Consultant 

Clinical Immunology & Allergy 

Child health department 

Sultan Qaboos University Hospital 

Oman   

 

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Chris Seroogy
Sent: Friday, April 25, 2008 8:45 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] Disseminated mycobacterium

 

Dear Colleagues:

 

I would like your advice on management of a 19 m/o previously healthy

caucasion girl who presented 4 days ago with thrombocyopenia and anemia.

Her bone marrow biopsy revealed numerous AFB+ organisms.  Her blood grew

mycobacterium and pneumococcus and her stool is growing mycobacterium.

Further identification is pending.  She has tremendous hepatospenomegaly and

high fevers.  Family history is incomplete as mother is adopted and parents

are unlikely related.  She is fully immunized (including live vaccines). ALC

2020, IgG and IgM elevated for age. She is being treated with a "cocktail"

of antimicrobials for mycobacterium per our ID team and vancomycin.  She

remains critically ill.

 

It seems likely that she has a defect in IFN-g/IL-12 axis.  We will be

sending blood to Steve Holland next week.  In the interim, I would like

opinions about using IFN-g (or perhaps IFN-a if this is a complete IFNR1

defect.)  Have any of you empirically used IFN-g in this setting?  Is there

any downside?  How rapid should improvement be observed if there is a

functioning IFNR?  Thank you for your insights, Chris

 

 

Chris Seroogy, M.D.

 

University of Wisconsin

 

Assistant Professor

 

Dept. of Pediatrics

 

Mail:  H4/474 CSC, Mailstop 4108

 

Shipping:  H4/431 CSC, Mailstop 4108

 

600 Highland Ave.

 

Madison, WI  53792

 

phone: 608- 263-2652

 

fax: 608-265-0164

 

 

 

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