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

[Chinen, Javier]

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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