[PAGID] please post

[Cunningham-Rundles, Charlotte]

This is as bad as it gets of course. I assume TPN is in place as that will
be needed. Don't forget the zinc in this replacement. Has Entocort been
tried? IV or other absorbable steroids are likely to be needed  and I assume
you have done that already. Our next addition would be 6MP.  (CMV is
negative?) 

I sent this  case to Lloyd Mayer too.

PS (These autoimmune things - all you note- are found in DiGeorge pts who
can be very hypogamma too so maybe don't discount that thought.)

Charlotte Cunningham-Rundles MD PhD
Departments of Medicine, Pediatrics
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue,
New York City, New York, 10029
212 659 9268 (phone)
212 987 5593 (fax)



> From: Jack R <jroutes at mcw.edu>

> Reply-To: PAGID <pagid at list.clinimmsoc.org>

> Date: Wed, 20 Jan 2010 08:08:58 -0600

> To: PAGID <pagid at list.clinimmsoc.org>

> Subject: [PAGID] please post

> 

> I am posting this unusual patient for a colleague at the University of

> Virginia ( Larry Borish <LB4M at virginia.edu>) who asked me to comment on this

> unusual patient----thanks

> 

> Jack Routes, MD

> Chief, Section of Allergy and Clinical Immunology

> Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics

> Department of Pediatrics

> Children's Hospital of Wisconsin

> Medical College of Wisconsin

> 9000 W. Wisconsin Ave.

> Milwaukee, WI  53226-4874

> 

> 

> "What do you think of a 17 year old kid who 3 years ago developed in no

> particular order: AIHA, autoimmune neutropenia, had his eosinophils

> disappear (AEC of 200-300 all his life to 0 for the past 3 years), and had

> his B cell disappear along with all immunoglobulins.  He is in the hospital

> dying of his enteropathy and secondary cachexia.  Other features are

> esophageal candidiasis, low testosterone (but nothing else hormonally

> including PTH), and maybe Menière's disease.  He has diffuse large nodular

> lung disease and impressive bronchiectasis but nothing I'd call ILD (also no

> thymoma).

> 

>   My real question is he is about to be the 2nd antibody deficient patient

> who has died on me in the past 2 years of refractory protein-losing

> enteropathy.  Gut shows excessive CD8s but no B cells.  I'm going to send

> you gut DNA for HHV8, what the heck.  Anyway have you ever successfully

> treated refractory enteropathy in an antibody-deficient patient?  I'm

> considering infliximab or cyclosporin but thought you might have a better

> idea"

> 

> 

> 

> 

>  

> 

>