[PAGID] please post

[dmvascon at usp.br]

Dear Jack

The first disease I would think would be ID with thymoma (Good's  
syndrome), that is commonly associated with autoimmunity,  
anaeosinophilia and sometimes anabasophilia, hypogamma with B cell  
disappearance and sometimes mucocutaneous candidiasis.
Despite the fact that this disease usually appears in the fifth or  
sixth decade of life I have already seen two patients presenting in  
the third decade, and in one of them the diagnosis of thymoma was  
undertaken in the necropsy, after approximately five years of  
follow-up and no evidence of thymoma in chest CT.
Another disease I would think about is ADA deficiency, that may appear  
lately in life, and even drug induced hypogamma (I've seen very severe  
cases induced by hidantoin and carbamazepine). I also think a lymphoma  
could be a possible differential diagnosis. Are these CD8 cells clonal?

Good luck with your patient.

Dewton




Citando "Routes, John" <jroutes at mcw.edu>:


> 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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