[PAGID] please post

[Bodo Grimbacher]

Dear Jack and Larry,
we have adult CVID patients with enteropathy and wasting but most of 
them respond to 0.5 to 1.0 mg of Prednisolone per kg BW. In only a 
handful we had to start Infliximab with good success and Infliximab was 
better than any DMARD.
However, I agree that this kid is different. I would support the 
previously mentioned suggestions to
a) exclude CMV
b) CD8 LGL (any splenomegaly?)
c) gut lymphoma (need to scope the whole small bowel with this new 
technique or do a capsule endoscopy)
Yours, Bodo
(Grimbacher, London)

> 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"

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