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

[Berger, Melvin]

Has her T-cell receptor spectratype been analyzed ? Maybe that, too, is oligoclonal. If so, it would make the oligoclonality of the antibodies less mysterious and less troubling. 
 
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 2:20 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Puzzling case of hypergammaglobulinemia with absence of antibody responses



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

> 

>  WARNING: E-mail sent over the Internet is not secure.

>  Information sent by e-mail may not remain confidential.

> 

>  DISCLAIMER: This e-mail is intended only for the

>  individual to whom it is addressed. It may be used only in accordance

> with applicable laws. If you received this e-mail by mistake, please

> notify the sender and destroy the e-mail.

> 

> 

> 

> 

> 

> 

> 

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Visit us at www.UHhospitals.org.

The enclosed information is STRICTLY CONFIDENTIAL and is intended for the use of the addressee only. University Hospitals and its affiliates disclaim any responsibility for unauthorized disclosure of this information to anyone other than the addressee.

Federal and Ohio law protect patient medical information, including psychiatric_disorders, (H.I.V) test results, A.I.Ds-related conditions, alcohol, and/or drug_dependence or abuse disclosed in this email. Federal regulation (42 CFR Part 2) and Ohio Revised Code section 5122.31 and 3701.243 prohibit disclosure of this information without the specific written consent of the person to whom it pertains, or as otherwise permitted by law.
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