[PAGID] patient who lacks long-term memory antibody responses
Eleonora Gambineri
eleonora.gambineri at unifi.it
Mon Jul 20 15:49:15 EDT 2009
Melvin,
no I didn't, but I could do. Although he only had one episode of
pneumonia so far.
How would you explain the CD4 deficit in this case?
Anyhow I will check. Thanks for suggesting.
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 20/lug/09, at 21:31, Berger, Melvin wrote:
> 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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> Visit us at www.UHhospitals.org.
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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.
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> 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
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