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

[Mel.Berger at cslbehring.com]

Is there evidence for chronic (subclinical) infection such as elevated ESR, CRP or WBc ?  Ig so, that might push towards careful study of her aireways, and use of IgG supplementation.

Mel Berger
________________________________
From: jag aggarwal [jag.aggarwal at gmail.com]
Sent: Friday, January 04, 2013 4:44 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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