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

[Richard Wasserman]

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> 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

> Fax:  504-896-9311

> Email:  kparis at lsuhsc.edu <mailto:kparis at lsuhsc.edu>

>

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>

> From: 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

> 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  Fax 310.267.009

>

>

> From: Alan Redding <aredding99 at gmail.com<mailto:aredding99 at gmail.com>>

> Reply-To: pagid listserve <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>>

> 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

> cell (404) 593-33338

> fax (404) 355-0079

>

>

>

>

>

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-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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