[CIS PIDD] [cis-pidd] Lymphopenia 17 year-old

[Soheil Chegini]

Hi Jason,
 
If a neonatal cardiac repair results in transection of the thoracic duct or elevation of the right atrial pressure, recirculation of lymphocytes can be impaired with loss of lymphocytes into the GI lumen and consequent lymphopenia, which in more severe cases can lead to full blown intestinal lymphangiectasia. The memory cells are lost, but frequently such patients can compensate by producing more naïve T-cells and maintaining the entire TCR repertoire. For that reason even at very low lymphocyte levels that in other cellular immunodeficiency disorders predispose to opportunistic diseases and require prophylaxis many of these patients do not display the same problems without antimicrobial prophylaxis.
 
A simple screening of fecal alpha-1 antitrypsin might provide some answers. Obviously, if this is the case T-cell in the peripheral blood would be expected to be naïve, and CD45 RA/RO phenotype which was previously mentioned would shed some light on this issue.
 
Best,
 
Soheil
 
Soheil Chegini, M.D.
Exton Allergy & Asthma Associates
656 West Lincoln Hwy.
Exton, PA 19341
Phone: (610) 269-3066
Fax: (610) 269-8615
 

________________________________
 From: Jason Raasch <jraasch at midwestimmunology.com>
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org> 
Sent: Friday, August 30, 2013 5:22 PM
Subject: [cis-pidd] Lymphopenia 17 year-old
  
All, 
The following is a brief summary of a patient my colleague now cares for.  [I hope the text formatting is forgiving]. 
** THE QUESTION: What is your opinion regarding pneumocystis prophylaxis based on his flow cytometry? ** 
17-year-old male with a history of congenital heart defect and cardiac repair at birth.  Thymus was likely removed with 1st surgery.   
Clinically doing very well.  Mild psoriasis. No significant infectious history.  Immunoglobulin levels are normal, CD 19 absolute and percentages  are normal. FISH analysis of DiGeorge was negative for 22q11 deletion at birth and has not been repeated.  
He has been noted to have lymphocytopenia with CD3 positive T cells consistently less than 500.  His recent CD3CD4 numbers have fallen due to a decrease in his absolute lymphocyte count and are reported as follows: 
    July 2013   June 2012    
CD3   46%  299/uL   43%   414/uL    
CD3CD4   34%  221/uL   30%   289/uL    
CD3CD8   19%  124/uL   24%   231/uL    
CD4/CD8 ratio   3.00   2.42      
   
CONVERSATIONS ABOUT THIS TOPIC SEEM TO GO AROUND AND AROUND.  JUST LIKE TO HEAR HOW SOME OF YOU MIGHT APPROACH THIS QUESTION. 
  
THANKS. 
  
-J

  
Jason Raasch, MD

Midwest Immunology Clinic
15700 37th Ave N, Ste 110
Plymouth, MN 55446

TEL: (763) 577-0008
FAX: (763) 577-0192 
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