[PAGID] Puzzling patient with high normal IgG levels but no antibody responses to vaccines
HOWARD M LEDERMAN
hlederm1 at jhmi.edu
Thu Jul 12 16:00:32 EDT 2007
No GI sxs at all, and no h/s megaly.
Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics and Medicine
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
e-mail: Hlederm1 at jhem.jhmi.edu
----- Original Message -----
From: "Routes, John" <jroutes at mcw.edu>
Date: Thursday, July 12, 2007 2:18 pm
Subject: Re: [PAGID] Puzzling patient with high normal IgG levels but no antibody responses to vaccines
To: pagid at list.clinimmsoc.org
> Does the baby have any abdominal pain, diarrhea or other localizing
> symptoms? Could be that the B cells are all hiding out in the abdomen/gut--
> I am still a bit suspicious of a B cell lymphoma. I would CT his
> abdomen and
> look for adenopathy.
> Jack
>
>
> John M. Routes, MD
> Chief, Section of Allergy and Clinical Immunology
> Professor of Pediatrics, Medicine, Microbiology and Medical Genetics
> Department of Pediatrics
> Children's Hospital of Wisconsin
> Children's Research Institute
> Medical College of Wisconsin
> 9000 W. Wisconsin Ave.
> Milwaukee, WI 53226
>
> Phone: 414-456-4803 Administrative Assistant
> Phone: 414-266-6840 Allergy Clinic
> Fax: 414-266-6437
> email: jroutes at mcw.edu
>
>
>
>
> From: HOWARD M LEDERMAN <hlederm1 at jhmi.edu>
> Reply-To: <pagid at list.clinimmsoc.org>
> Date: Thu, 12 Jul 2007 13:22:11 -0400
> To: PAGID LISTSERV <pagid at list.clinimmsoc.org>
> Cc: "Guerrerio, Pamela A" <pfrisch1 at jhmi.edu>
> Subject: [PAGID] Puzzling patient with high normal IgG levels but no
> antibody responses to vaccines
>
> I have seen pts with low normal IgG levels but poor antibody
> responses, but
> this case seems to be way out of my experience. Are there any specific
> diagnostic tests that I should be considering?
>
> --------- ----------- ------------- ------------ -------------- ------------
> ---------- -------------
>
> J.S. is a 10-month-old Caucasian male who had no significant infections
> until 8 mos of age when he developed fever (T=104), lethargy and poor
> appetite. A full sepsis workup was conducted and his blood culture grew
> Pseudomonas aeruginosa in 24 hrs. His left tympanic membrane spontaneously
> ruptured during his hospitalization and cultures also grew
> pseudomonas. A
> head CT was normal except for bilateral middle ear opacification. A
> chest
> x-ray was normal. An abdominal ultrasound was normal.
>
> An immunodeficiency workup showed virtually no CD19+ B-cells (0-2%;
> 11-169/cu mm) with normal numbers of T (90% CD3, 68% CD4 = 4467/cu
> mm, 21%
> CD8 = 1349) and NK (6% = 368/cu mm) cells. Serum immunoglobulins were
> normal
> for age (IgG 446 mg/dL, IgA 30 mg/dL, IgM 35 mg/dL).
>
> J.S. had no other history of infections other than intermittent mild
> viral
> upper respiratory tract symptoms. He has had no skin infections or urinary
> tract infections. His growth has been normal. He had received all routine
> childhood vaccines.
>
> Subsequent lab tests have shown INCREASING IgG levels (1020 mg/dL)
> with low
> normal IgA (34 mg/dL) and IgM (31 mg/dL). Despite the elevated IgG levels,
> he had NO detectable IgG antibody to previously administered standard
> vaccines (<0.2 mcg/ml to 14 tested pneumococcal serotypes, < 0.11 to
> HIB
> mcg/ml and <0.10 IU/ml to tetanus). He had NO increase in IgG Ab after
> booster doses of Prevnar and Hib conjugate vaccines; tetanus
> increased only
> marginally to 0.66 IU/ml. Repeat T and B cell studies by FACS were
> essentially the same (CD19 3%; CD20 3.3 %; abs ct 140). Serum IFE
> showed no
> evidence of a monoclonal gammopathy. PCR tests for EBV and CMV were
> negative.
>
> I am open to any and all suggestions.
>
>
>
> Howard
> Howard M. Lederman, M.D., Ph.D.
> Professor of Pediatrics and Medicine
> 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
> e-mail: Hlederm1 at jhem.jhmi.edu
>
>
>
>
More information about the PAGID
mailing list