[CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

[Church, Joseph]

I too would treat.  Although she responded to protein immunogens (I would venture these titers will drop over the next few months), she failed to respond to both pure and protein conjugated polysaccharide vaccines.

Joe Church
Children's Hospital Los Angeles

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Richard Wasserman
Sent: Thursday, May 03, 2012 12:18 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

Of course I agree with Mary Ellen and Marc but the key question is not how likely she is to do well but what is the likelihood that her next pneumonia will leave her with irreversible lung disease. I agree with Ken, I'd treat.
Richard Wasserman
On Thu, May 3, 2012 at 2:13 PM, Paris, Kenneth <kparis at lsuhsc.edu<mailto:kparis at lsuhsc.edu>> wrote:
Alan,

I would agree with Marc.  We have had the exact situation in a few patients here.  When they have elected to watch and wait (due to a perceived state of "wellness"), they eventually do get ill and have required IgG replacement to regain their health.  It seems your patient has already had a pneumonia which was likely a result of her antibody deficiency.  Perhaps other innate immune mechanisms have kept some mild/recurrent infections at bay up until now, but a severe infection (pneumonia/sepsis/meningitis) would be an unfortunate consequence of treatment refusal.  There won't be a consensus among the group as to the "correct" thing to do, but with a hypogam as profound as you describe I would treat her.

Ken

Kenneth Paris MD, MPH
Assistant Professor of Pediatrics
A/I Fellowship Training Program Director
Division of Allergy and Immunology
LSU Health Sciences Center
Children's Hospital of New Orleans

Mail:
200 Henry Clay Avenue
Children's Hospital
Research Institute for Children 4th Floor
New Orleans, LA 70118
Phone:  504-896-9589<tel:504-896-9589>
Fax:  504-896-9311<tel:504-896-9311>
Email:  kparis at lsuhsc.edu<mailto:kparis at lsuhsc.edu> <mailto:kparis at lsuhsc.edu<mailto:kparis at lsuhsc.edu>>

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From: pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org> on behalf of Riedl, Marc
Sent: Thu 5/3/2012 12:50 PM
To: pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?



We follow a number of similar patients at our center.  We attempt to gather as much immunologic data as we can including switched memory B-cell profile and vaccine responses.  However, at the end of the day, this is probable CVID and it's a matter of discussing the risks and benefits of Ig replacement therapy with the patient.  I typically get chest CT and PFTs with DLCO to help guide the strength of that recommendation.  At these levels and with a history of pneumonia, I strongly encourage patients to start therapy given the potential risk of the next infection being serious and rapidly progressive.  That said, we have patients that with understanding of the risk elect to forgo IVIG ("I've done fine to this point") and we monitor them clinically over time.  Some have in fact done well for many years without evidence of recurrent infection or pulmonary consequences.  It would tremendously useful to have methods of risk-stratification for these patients, but aside from the above diagnostics, I think we're making only educated guesses about the optimal treatment course.

Best,

Marc

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<tel:310.206.4345>  Fax 310.267.009


From: Alan Redding <aredding99 at gmail.com<mailto:aredding99 at gmail.com><mailto:aredding99 at gmail.com<mailto:aredding99 at gmail.com>>>
Reply-To: pagid listserve <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org><mailto:pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>>
To: pagid listserve <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org><mailto:pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>>
Subject: [CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

      Recently, an internist referred a 54 yo F to me because her total protein (TP) level was low (5.8 g/dL) and her gamma globulin fraction was low (0.2 g/dL).  Bloodwork was done as part of a routine physical.  In her twenties, while pregnant, she says that she was hospitalized for pneumonia (patient doesn't know details of this infection).  Since then, she says that she has been treated for pneumonia twice as an outpatient, but she cannot recall having a CXR on either occasion.  This is her only infectious history.  Other than hypercholesterolemia, she is healthy.  She feels perfectly fine.  No history of recurrent sinusitis, bronchitis, cough, etc.  She even asked me "Why am I here?"
      On further workup, total Ig A was undectectable (<4 mg/dL), IgM was low at 23 mg/dL, and IgG was low at 240 mg/dL.  She had protective levels to tetanus (0.45 IU/mL) and diptheria (0.07 IU/mL), which increased after Tdap vaccination to 1.85 mg/dL and 0.20 mg/dL, respectively.  She also had protective antibody levels to Varicella Zoster virus.  She did not respond to the first dose of hepatitis A virus vaccine, but did show "reactive" antibody levels after receiving the second dose of hepatitis A virus vaccine.  However, she showed zero response to Pneumovax vaccine, the H. flu vaccine, or the meningoccal polysaccharide vaccine.
    In summation, it appears that she can mount an immune response to protein antigens, both new and old.   However, since she did not respond to the H. flu conjugate vaccine, and, she did not respond to the hepatitis A vaccine until after the second dose, the response may be sluggish.   And, she cannot respond to new polysaccharide vaccines.
         I have never seen a patient like this, before.  Could it be that I have just caught CVID, and she is just lucky that she has not had a serious infection?  Or, might one say, "Well, she does have low antibody levels.  But, something must be working right, because she is 54, and hasn't had frequent or severe infections.   It may be difficult to talk her into starting immunoglobulin replacement when she feels normal, and has hardly been sick.   However, I want to recommend the safest course of action, both for her sake, and, for mine.  I would appreciate any recommendations, especially, if anyone has ever had personal experience with patients such as this.

Sincerely,
Alan Redding, M.D.
Redding Allergy and Asthma Center
3193 Howell Mill Rd. NW, Ste 102
Atlanta, GA 30327
direct line (404) 941-1183<tel:%28404%29%20941-1183>
cell (404) 593-33338
fax (404) 355-0079<tel:%28404%29%20355-0079>





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DallasAllergyImmunology
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