[PAGID] Puzzling patient with high normal IgG levels but no antibody responses to vaccines

[Routes, John]

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?

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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




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