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

[Patsy Giclas]

Is her complement normal?  CH50 and AH50, lectin pathway?
Can she make an IgM response to a novel antigen?

---------------------------------------------------------
Patricia C. Giclas. Ph.D.
Director, Complement Laboratory
Advanced Diagnostic Laboratories
Professor, Pediatrics Dept,  Allergy and Immunology Division
National Jewish Health
1400 Jackson St., Denver, CO 80206 U.S.A.

Office: D409, Neustadt Building
Phone: 303-398-1217
Fax: 303-270-2128
Email: giclasp at njhealth.org



From: jag aggarwal <jag.aggarwal at gmail.com>
Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Date: Fri, 4 Jan 2013 12:37:53 -0700
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Subject: [cis-pidd] Possible CVID case

 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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