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

[Nacho Gonzalez]

I think that checking memory B cells is a great idea, but it is quite
unspecific, as you can see absence of memory B cells in CVID, Hyper IgE and
Hyper IgM... I heard one patient from The Netherlands (Esther de Vries) with
a similar clinical picture, and as far as I know the patient improved with
IVIG/SCIG.  Perhaps she could help you

Nacho. Immunodeficiencies Unit. Hospital 12 octubre. Madrid. Spain.


2009/7/17, Chinen, Javier <jxchinen at texaschildrens.org>:

>

>  One of my Hyper IgE patients (STAT3 mutation confirmed) and another with

> a not identified genetic syndrome (extensive work up done) have the same

> phenotype. The HIES patient was on IVIG, now he is well with just antibiotic

> prophylaxis, decisions on IVIG were made based on family comfort with the

> potential risk of overwhelming infection and access to medical care. The

> second patient is OK with antibiotic prophylaxis (Bactrim in both cases).

>

> Lack of memory humoral responses is reported in HIES. It would be

> interesting to check CD27 B cells.

>

> Javier

>  ------------------------------

>

> *From:* pagid-bounces at list.clinimmsoc.org [mailto:

> pagid-bounces at list.clinimmsoc.org] *On Behalf Of *Howard M Lederman

> *Sent:* Thursday, July 16, 2009 1:50 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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