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

[Jack Bleesing]

An no B-cells using CD20 or other pan B-cell markers. 

T-cell system/function?

B-cell progenitors in bone marrow?

JB

---------------------------------------------------------------------------
Jack J.H. Bleesing, M.D., Ph.D.
Cincinnati Children's Hospital Medical Center
Division of Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org
http://www.cincinnatichildrens.org/immunodeficiencies/



>>> hlederm1 at jhmi.edu 7/12/2007 4:00:32 PM >>>

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 

>  

>  

>  

>