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

[Seppänen Mikko]

Dear Jag,

I would love to hear as well whether she is able to make IgE (lacks in appr. 90%? of CVIDs). Also, what are the actual titers (and the method to assess them), do YOU define protective titers as >0.35 (which many pediatricians also in the US follow), >0.65 (which best differentiated in British-Finnish material), or like suggested in the US Practice Parameter >1.3 (or some other threshold)?

And like all others, whether there is bronchiectasis or other PAD associated conditions (or also emphysema and nasal polyposis as well to suggest MHCII def)? Smoker? Asthma and corticosteroids p.o. against this (would be the most common cause in our hospital)?

Are there necrotic skin infections or like (??) to suggest MyD88/IRAK4 (and low mBs and subclasses due to those?), detectable (?) total complement defs (other than MBL), anything to suggest SGD (hyposegmented neutrophils?low chemotaxis? skin infections)?, hyposplenism (acquired/primary?) /Howell-Jolly in smear, is either nasal Ex-NO,  saccharine test normal or cilia biopsy is normal (? 4 pneumonias + recurrent sinusitis and normal specific antibodies in a youngish woman would be a cause to exclude ciliary dyskinesias )....

....we are left with the occasionally occuring IgG (IgG2)+/-IgA +/- IgM, low, smB low patient with normal responses. There is no consensus on how to treat these (in this PAGID list or elsewhere), they might represent an independent subset of PADs or CVID in the making (follow up at least, also the specific titers). I have a few such patients, I have personally chosen to treat IF ab prophylaxis does not keep the pneumonias away and IF there IS bronchiectasis (but physiotherapy important as well) or rapidly deteriorating LFTs in follow up. 

Also, if most serotypes are <0.65, I would first vaccinate with Pneumovax and definitely check the responses, severe local reactions would then be rare (seen just one in >15 years). 

Hope this helps, 
Yours

mikko seppänen, md phd, assistant prof
Finland



________________________________________
Lähettäjä: jag aggarwal [jag.aggarwal at gmail.com]
Lähetetty: 4. tammikuuta 2013 21:37
Vastaanottaja: CIS-PIDD
Aihe: [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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