[PAGID] please post

[Waleed Alherz]

This is very interesting since I have a similar patient. 

16 years old with history of AIHA & thrombocytopenia progressive hypogamma (but normal B cell count) and bronchiectasis. I have diagnosed his as CVID. He has been having chronic diarrhea and lost 12 kg. Upper & lower scopes showed esophageal candidiasis, lymphocytic colitis (T cells with no plasma cells), no granuloma or evidence of IBD, no evidence of viral infection neither by staining or PCR studies. 

According to our GI specialist, lymphocytic colitis is a disease that can occur in CVID. The patient is on steroids and we will start him on methotrexate. It is early to see a response.


 

Waleed Al-Herz, MD

Allergist & Clinical Immunologist

Al-Sabah Hospital

Kuwait

 

 

> From: jroutes at mcw.edu

> To: pagid at list.clinimmsoc.org

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

> 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"

> 

> 

> 

> 

> 

> 

> 

 		 	   		  
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