[PAGID] patient who lacks long-term memory antibody responses

[Berger, Melvin]

Ele- Have you checked complement in this one ?
 
Melvin Berger, M.D., Ph.D.
Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Eleonora Gambineri
Sent: Mon 7/20/2009 11:27 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] patient who lacks long-term memory antibody responses


Dear Howard, 

I am following up a 8 yo boy with similar clinical presentation than yours. He has been suffering from recurrent/chronic sinusitis since a couple of year (before he was healthy). Recently he also suffered from pneumonia. 

Hereby his labs:

CBC and differential: normal
LFTs: normal
Creatinine: normal
 
Vaccinal antibodies:
Anti-tet: neg  (last booster 2 yrs ago)
Anti-measles: neg
Anti-dift: neg
Anti-HIV: neg
Anti-pneumococcus: ongoing
 
CMV Ab: IgG POS;               PCR CMV (peripheral blood): neg
EBV Ab: neg;                         PCR EBV (peripheral blood): neg
 
 
IgG 1240 mg/dl
IgA 166
IgM 179
 
IgE 34 kU/L
 
IgG1   1070 mg/dl
IgG2    140
IgG3    88
IgG4    2
 
Anti-nuclear Ab: neg

His T cell phenotype showed low CD4 count (400-500/ul) mainly CD45 RO memory CD4 T cells (65%). The B cell phenotype showed a normal differentiation of memory B cells except for increased transitional B cells (9,8%).

He is at the moment in prophylaxis with Azithromycin: 350 mg 3 times/week. No infections so far.


Before considering IVIG I will see how he will do clinically. If he need to stay on antibiotics to be free from infections I will probably consider immunoglobulin replacement. 


Since the thymic output seems normal (most of CD4+ CD45RA+ cells are CD31+) I was also considering other condition with helper T cell-dependent antibody defects i.e. XLP ? Although I am also open to any kind of suggestions!!


Best wishes,
Ele


*******************************************************************
Dott.ssa Eleonora Gambineri
Ricercatore Universitario
Universita' degli Studi di Firenze
Dipartimento di Pediatria-Ospedale Pediatrico "Anna Meyer"
Viale Gaetano Pieraccini, 24
50139 FIRENZE
Tel 055 5662405 (ufficio)
       055 5662464 (lab)
Fax 055 4221012
e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it

Eleonora Gambineri, MD
Researcher/Assistant Professor
University of Florence
Department of Pediatrics-"Anna Meyer" Children's Hospital
Viale Gaetano Pieraccini, 24
50139 FIRENZE
ITALY
Tel +39 055 5662405 (office)
       +39 055 5662464 (lab)
Fax +39 055 4221012
e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it
********************************************************************





On 19/lug/09, at 03:08, Berger, Melvin wrote:


	C4 deficiency causes the inability to make memory responses to T-dependent antigens. Each time they are immunized they make a repeated primary response, but even after several immunizations there is still only a little IgG, and the amount doesn't increase. This was well documented by Hans Ochs in a New england journal paper about 20 yeatrs ago (or more) with phage. Evebtually, the patients' IgG drops- this also occurs in C3 deficiency.  I would definitely check C3 and C4 if you don't have those results. 
	
	On the IGIV issue, he does seem to maintain Abs against quite a few antigens. I might try prophylactic antibiotics before an empirical trial of IgG supplementation if the former is not enough. If there is an outbreak of measles, he should probably be given a dose of IgG.
	
	Melvin Berger, M.D., Ph.D.
	Adjunct Professor of Pediatrics and Pathology
	Case Western Reserve University
	phone 216 844 3237
	
	Director, Jeffrey Modell Center for Primary Immune Deficiencies
	Division of Allergy-Immunology
	Rainbow, Babies and Children's Hospital
	University Hospitals of Cleveland
	RB&C Rm 504, MS 6008B
	11100 Euclid Ave.
	Cleveland, OH 44106
	
	________________________________
	
	From: pagid-bounces at list.clinimmsoc.org on behalf of Howard M Lederman
	Sent: Thu 7/16/2009 2:49 PM
	To: PAGID LISTSERV
	Subject: [PAGID] patient who lacks long-term memory antibody responses
	
	
	
	I have a fascinating  12 yr old male patient with a history of recurrent infections (mostly upper and lower respiratory tract).  No individual infection has been overwhelmingly severe, but the sum of all infections leads to a significant number of school absences.
	
	He has low IgG and IgM levels with normal IgA levels.  He makes normal IgG antibody responses to a wide variety of vaccines, but does not seem to be able to maintain long-term immunity.  (See attached spreadsheet.  Sheet 1 tabulates the antibody and immunglobulin levels; sheet 2 lists the immunizations.)
	
	Is this a pattern that others have seen?    I am open to any ideas for further evaluation.  Would anyone offer this child gamma globulin? 
	
	
	Howard
	Howard M. Lederman, M.D., Ph.D. 
	Professor of Pediatrics, Medicine and Pathology 
	Division of Pediatric Allergy and Immunology 
	Johns Hopkins Hospital - CMSC 1102 
	600 N. Wolfe Street 
	Baltimore, MD 21287-3923 
	Phone: 410-955-5883 
	Fax: 410-955-0229 
	Email: Hlederm1 at jhem.jhmi.edu 
	
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The enclosed information is STRICTLY CONFIDENTIAL and is intended for the use of the addressee only. University Hospitals and its affiliates disclaim any responsibility for unauthorized disclosure of this information to anyone other than the addressee.

Federal and Ohio law protect patient medical information, including psychiatric_disorders, (H.I.V) test results, A.I.Ds-related conditions, alcohol, and/or drug_dependence or abuse disclosed in this email. Federal regulation (42 CFR Part 2) and Ohio Revised Code section 5122.31 and 3701.243 prohibit disclosure of this information without the specific written consent of the person to whom it pertains, or as otherwise permitted by law.
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