[PAGID] please post

[Ballas, Zuhair]

I had a similar adult patient a few years back with massive CD8 infiltration in the gut. That particular patient responded well to interferon alpha therapy.
My suggestion is to go over the intestinal biopsy with the pathologist. If there are granulomas then TNF antagonists might be worth a shot. If there are no granulomas, you may want to try interferon.

Zuhair


-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Kate Sullivan
Sent: Wednesday, January 20, 2010 8:15 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] please post

Very very tough.  The adult folks probably see more but I am a big fan  
of Rapamycin for T cell infiltrates in the gut.  We also recently had  
a theoretical discussion on a similar patient at CHOP where we talked  
about BMT.

Kate
On Jan 20, 2010, at 9:08 AM, Routes, John wrote:


> 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"

>

>

>

>

>

>

>


Kathleen Sullivan MD PhD
Professor of Pediatrics
Chief, Division of Allergy Immunology
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 267-426-0363