[CIS PIDD] Patient with thymoma, autoimmunity, recurrent infections, normal Igs and B cells

[Juan Carlos Aldave Becerra]

Dear Yesim,
Thank you for your response.Currently we don't perform pneumococcal titers in Peru.I will look for CF.
Thank you very much,

Juan Carlos Aldave, MD
Allergy and Clinical Immunology
Rebagliati Martins National Hospital - Lima, Peru
Primary Immunodeficiency Committee of the Latin American Association of Immunology
WAO Special Committee on Eosinophil and Mast Cell Disorders
Clinical Immunology Committee of the IUIS

CC: pagid at list.clinimmsoc.org
From: dryesimyilmaz at gmail.com
Date: Mon, 23 Jul 2012 09:58:05 -0400
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] Patient with thymoma, autoimmunity,	recurrent infections, normal Igs and B cells

Hi Juan Carlos, Has CF been ruled out? What about pneumococcal titers? I would check vaccine response. Yesim 
Yesim Yilmaz Demirdag West Virginia University School of Medicine Morgantown, WV 

Sent from my iPhone
On Jul 23, 2012, at 12:35 AM, Juan Carlos Aldave Becerra <jucapul_84 at hotmail.com> wrote:





Dear Doctors,
Last week I have evaluated a 8 year old boy with the following features:
1. Recurrent infections:- Many pneumonias and upper respiratory infections, once complicated with pleural effusion- Urinary tract infectionsEnviar- Giardia lamblia infections.- No results of cultures available (seen in other hospitals).- No other opportunistic infections.
2. Autoimmune features:- Diarrhea for several months (colitis, probable autoimmune?)- Autoimmune hepatitis (pending biopsy result).
3. Thymoma, diagnosed some months ago, surgery is planned.
4. Laboratory results (available analysis in Peru):- CBC, IgG, IgA, IgM, CD19, CD4, CD8, CD16/CD56, CH50, NBT: normal values- IgE: 600- Antibody to tetanus: <0.1 (although he has received 3 doses according to vaccination schedule)- Antibody to HBsAg: negative (although he has received 3 doses according to vaccination schedule)- Antibody to rubella: negative (although he has received 1 dose according to vaccination schedule)
5. The patient has a brother with similar characteristics and a healthy sister
I have revised the literature, I don't find any suggestive diagnosis except for CVID (although normal B cells and normal IgG, IgM and IgA values).
My questions are:Do you suggest any other diagnosis?Do you think he should receive IVIG?Thymoma should be removed?

Thank you so much,

Juan Carlos Aldave, MD
Allergy and Clinical Immunology
Rebagliati Martins National Hospital - Lima, Peru

 		 	   		  
 		 	   		  
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