[CIS-PAGID] Asymptomatic hypogammaglobulinemia

Charlotte cunningham-rundles charlotte.cunningham-rundles at mssm.edu
Thu Dec 30 18:58:19 EST 2010


I agree with Marguerite, that those with no Ig to mention and no antibody
can have very severe infections as an outcome -- have seen a few that
someone sat on for a while. So, of course I'd treat them myself too.



Lengkeek, Marguerite S. LCDR


> Have you checked for any secondary loss of protein/immunoglobulin? Does he

> have any GI symptoms that might cause a protein losing enteropathy? Have

> you considered ordering stool alpha 1 anti-trypsin and urine protein or

> UPEP?

>

> The other cases with the almost absent Ig run the risk of their first

> infections being severe ones. I follow 2 CVID patients that were

> misdiagnosed until their 60s. One was picked up after H. flu meningitis with

> gross pus from his spinal tap and a week in the ICU comatose on a vent. The

> other was picked up after S. pneumoniae sepsis. They did have infections

> (sinusitis and PNA) previously, so they may be different.

>

> Sincerely,

>

> Marguerite S. Lengkeek, MD

> LCDR, MC, USN

> Division Head, Allergy and Immunology

> Naval Medical Center San Diego

> Naval Training Center

> 2051 Cushing Rd.

> San Diego, CA  92106

> (619) 524-1519

> Marguerite.lengkeek at med.navy.mil

>

>

> -----Original Message-----

> From: pagid-bounces at list.clinimmsoc.org

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Charlotte

> cunningham-rundles

> Sent: Thursday, December 30, 2010 11:55 AM

> To: PAGID

> Subject: Re: [CIS-PAGID] Asymptomatic hypogammaglobulinemia

>

> Hi Joe,

>

>

> There are a lot of these around. We did a survey of our referrals for a 2

> year block and came up with over 100 --- referred with just this question.

> On paper, with pretty good antibodies, not CVID exactly. For insuracne, you

> can use that term I guess, but as for treatment, as others noted, maybe Ig

> is not currently needed. If he came to me, I¹d do a bunch more antibodies

> to firm up the current info, and follow yearly or more often if infections

> became an issue.

>

> PS: with regard to other exchanges: Some CVID subjects, with virtually no

> immune globulin, just do not have any infections. It is a real mystery, but

> it is a real fact. I have one like the one Andy mentioned, an engineer of

> at least 60 now, with IgG of about 150, no IgA or M, with NO antibody and NO

> infections. Sure, he is a CVID, but he refuses Ig, and has done for 15 or 20

> years.

>

> PSS: have a wonderful New Year!

>

>

> Charlotte

>

> Church, Joseph

>

>

>

> Colleagues:

>

> I am seeing a 42yo man with no significant infection history. A low

> globulin fraction on a chem. panel lead to his primary MD to obtain QUIGs:

> IgG 309, IgA <7, IgM 30. He has protective tetanus and Hib antibody, but

> responded to only 3 of 23 serotypes following Pneumovaxtm.

>

> Review of past chem. panels suggest these finding have been present

> for 10 years.

>

> Glaum and Levinson reported similar patients (Ann Allergy Asthma

> Immunol 2008;100:396-7), but I could not get a sense of what they did about

> their findings.

>

> Should patients like this be placed on immunoglobulin replacement?

>

> Joe Church

> Children¹s Hospital Los Angeles

>

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