[CIS PIDD] [cis-pidd] Possible CVID case

[Riedl, Marc]

This is a relatively young woman with 4 pneumonias (assuming documented true pneumonia), one resulting in critical illness.  Lacking another identifiable underlying cause, the hypogamm, though not profound should be treated in my opinion.  Chest CT shows ???.  I'd be concerned about structural lung disease due to recurrent pneumonia.

Marc Riedl, M.D., M.S.
Associate Professor of Medicine
Section Head, Clinical Immunology and Allergy
UCLA - David Geffen School of Medicine
10833 Le Conte Ave, 37-131 CHS
Los Angeles, CA  90095-1680
Tel 310.206.4345  Fax 310.267.0090


From: <Saxon>, Andrew Saxon <asaxon at mednet.ucla.edu<mailto:asaxon at mednet.ucla.edu>>
Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Date: Friday, January 4, 2013 1:48 PM
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Subject: RE: [cis-pidd] Possible CVID case

I would be interested in knowing the total IgE level and major allergen specific IgE levels. Generally, they is quite low in CVID and if she has true seasonal allergies, the secondary obstruction may be a major factor in her sinus disease with increased risk of pneumonia. Also, Her sinus CT shows ???

Andy Saxon, MD
UCLA

From: jag aggarwal [mailto:jag.aggarwal at gmail.com]
Sent: Friday, January 04, 2013 1:45 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Possible CVID case

@Richard:  She has protective titres to 15 out of 23 serotypes of S. pneumo.  I was hesitant to reimmunize her with Pneumovax.
On Fri, Jan 4, 2013 at 2:27 PM, Richard Wasserman <drrichwasserman at gmail.com<mailto:drrichwasserman at gmail.com>> wrote:
The patient is below the lower limit of normal for all three major isotypes which would meet the classic definition of CVID. You don't say if the pneumococcal titers showed an increase post vaccination. The patient may have partially or fully intact memory but not be responsive to a neoantigen. It is very likely that IgG therapy would be beneficial.
Richard Wasserman
Dallas

On Fri, Jan 4, 2013 at 1:37 PM, jag aggarwal <jag.aggarwal at gmail.com<mailto:jag.aggarwal at gmail.com>> wrote:

 I have a 45 y/o female with recurrent sinus infections and four hospitalizations for pneumonia in the last 8 years. Each episode progressively getting worse. Most recent one requiring admission to ICU and was subjected to intensive infectious disease workup. She has no GI problems and no diagnosed autoimmune conditions. She does have significant seasonal allergies

Immune work up shows IgA 47 (n 81 - 463 ), IgG 615 ( n 694 - 1618 ), IgM <5 ( N 48 - 271 ), Low IgG2 at 142 ( N 241 - 700 )

Antibody titres to Tetenus Normal, antibody titres to S pneum protective range for 15/23 serotypes.

Mitogen stimulation to PHA, CON A, PWM normal. Lymphocyte Antigen Screen stimulation indices for Tetenus, Candida and PPD normal.

Lymphocyte subset panel all indices in normal range.

Immune assessments of B cell subsets (Mayo clinic ) shows: CD19 normal at 11 % , CD27 low at  4% ( N 6.3 - 52.8% ), CD27+ IgM- IgD- low at 1.5% ( N 2.3 - 28%),CD 27+ IgM+ IgD- only 0.3% ( N 0.0 - 5.3 % ), IgM+ % of CD19+ B cells high at 82.7% (N 26.0 - 78.0 % ), CD21+  normal at 98.7% of CD19 B cells, CD21- % of CD19+ B cells normal  at 1.3% all the other indices from Immune assessment of B cells are normal.
To me it appears that she fits into MB0 category of Paris classification and for the Euro classification group B+ smB- with CD21,norm.

Are we looking a t a new form of Immunedeficiency which si defiend by low numbers of switched memory cells with borderline low levels of IgG and almost absent IgM ?

I have two questions:
1.  Does she fit into the cate4gory of CVID
2. Would she benefit from Immunoglobulin replacement therapy even though her antibody titers to Tetenus and S. pneum and all the stimulation indices are normal and her IgG is really not very low although she has low IgA and her IgM level is below the detectable level for the lab.

Thanks

Jag Aggarwal MD
Private practice
Ped and Adult Alelrgy Immunology

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Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
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