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

[jag aggarwal]

 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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