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

[dmvascon at usp.br]

Dear Dr. Aggarwal 

I agree with Dr. Saxon view of a possible allergy influencing negatively for the susceptibility of infection. Is there any temporal relation between the infections and the season of deterioration of her respiratory allergy? 
She has IgG2 deficiency (partial) without evident deficit of the polysaccharide antibody response. We sometimes observe IgA deficient patients with a B cell phenotype similar to your patient, without any severe infection. 

Therefore I would try to treat her aggressively for her allergy and observe the evolution of upper and lower respiratory infections before thinking in IVIg replacement. 

Best regards, 

Dewton Vasconcelos 
University of São Paulo School of Medicine 

----- Mensagem original -----


> De: "Marc Riedl" <MRiedl at mednet.ucla.edu>

> Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org>

> Enviadas: Sábado, 5 de Janeiro de 2013 0:33:31

> Assunto: Re: [cis-pidd] Possible CVID case



> 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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