[PAGID] Puzzling case of hypergammaglobulinemia with absence of antibody responses

[Howard M Lederman]

Mel,

I don't know, but the most recent mitogen assays were normal so I don't imagine that IL-2 would do much

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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----- Original Message -----
From: "Berger, Melvin" <Melvin.Berger at UHhospitals.org>
Date: Wednesday, December 24, 2008 10:37 am
Subject: Re: [PAGID] Puzzling case of hypergammaglobulinemia with absence of antibody responses
To: pagid at list.clinimmsoc.org



> Howard-  What happens if you add IL-2 to the lymphocyte proliferation 

> assays ? 

>   

>  Melvin Berger, M.D., Ph.D.

>  Professor of Pediatrics and Pathology

>  Case Western Reserve University

>  phone 216 844 3237

>   

>  Director, Jeffrey Modell Center for Primary Immune Deficiencies

>  Division of Allergy-Immunology

>  Rainbow, Babies and Children's Hospital

>  University Hospitals of Cleveland

>  RB&C Rm 504, MS 6008B

>  11100 Euclid Ave.

>  Cleveland, OH 44106

>  

>  ________________________________

>  

>  From: pagid-bounces at list.clinimmsoc.org on behalf of Howard M Lederman

>  Sent: Wed 12/24/2008 10:20 AM

>  To: PAGID LISTSERV

>  Subject: [PAGID] Puzzling case of hypergammaglobulinemia with absence 

> of antibody responses

>  

>  

>  

>  I am evaluating a 20 month old girl with a perplexing combination of 

> findings, and could use some help:

>  

>  She was a full-term infant who began having recurrent otitis media at 

> 10 months of age. For unclear reasons, she was treated with 5 days of 

> prednisone + antibiotics for each OM.  At age 12 months, she stopped 

> standing, putting weight on her legs, and cruising.  She had normal 

> brain MRI, EMG, and nerve conduction velocity tests.  Shortly after, 

> she was treated with Decadron on 3 occasions within 2 wks for croup.  

> The following week, she developed stridor, and was intubated with 

> findings of laryngeal and subglottic swelling.  She was treated with 

> pressors, blood transfusion, and GM-CSF.  During her hospitalization, 

> a trachea culture was positive for Pseudomonas and Enterobacter.  

> Blood cultures were negative.  She developed candidal esophagitis and 

> C. difficile diarrhea. During her hospitalization, she was found to 

> have hepatosplenomegaly, still of unknown etiology. She was found to 

> have a drenal insufficiency, presumed to be secondary to steroid use, 

> and was started on replacement therapy.

>  

>  While hospitalized in 05/2008, she was evaluated for 

> immunodeficiency. She had normal immunoglobulin levels (IgA 291 mg/dL, 

> IgG 1270 mg/dL, and IgM 372 mg/dL).  She had normal adenosine 

> deaminase (67.3 nmol/h/mg) and purine nucleoside phosphorylase (1730 

> nmol/h/mg) levels.  She had diminished lymphoproliferative responses 

> to PHA (33% normal control), concanavalin A (6% normal control), and 

> pokeweed mitogen (58% normal control).  She had low percentages and 

> numbers of CD3 (31%; 294/cu mm), CD4 (24%; 223/cu mm) and CD8 (7%, 

> 65/cu mm) T-lymphocytes.

>  

>  When I first saw her last month, she had normal responses to T-cell 

> mitogens (unstimulated 318 cpm, Phytohemagglutinin A 77,979 cpm, 

> Concanavalin A 29,606 cpm), and negative FISH for 22q11 deletions.  

> She had a borderline low white blood count (5850/cu mm) with 63% 

> lymphocytes (3710/cu mm).  She had low percentages of CD3 (35%), CD4 

> (29%; 998/cu mm) and CD8 (5%) T lymphocytes.  Despite the high levels 

> of IgG (and IgA), she had no detectable IgG Ab to previously 

> administered vaccines, nor to a subsequent booster dose of Prevnar.   

> PCR tests for HIV, CMV and EBV were negative.  Ferritin normal (17 

> ng/mL), trigylcerides slightly high (169 mg/dL).  Her IgG and IgA 

> levels have increased.  IFE shows multiple bands of restricted 

> electrophoretic mibility in IgG, IgA, kappa and lamda lanes

>  

>  

>  I am anxious for ideas to explain all of this.

>  

>  

>  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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