[PAGID] please post

[Routes, John]

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"