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

[Conley, Mary Ellen]

As Marc says, I don't think this kind of patient is all that unusual.  I do agree that she probably has evolving CVID.  If she doesn't want to start gammaglobulin, I would try to make a deal with her.  If she has a pneumonia (or other significant infection) in the next 2 years, she should start the gammaglobulin.  Sometimes patients like this do very well on prophylactic antibiotics alone for long periods of time.  

Mary Ellen Conley, MD
West Research Tower
LeBonheur Children's Hospital
50 N. Dunlap St.
Memphis TN  38103-2800
Tel     901-287-4657
FAX  901-287-4551
mconley at uthsc.edu<mailto:mconley at uthsc.edu>


-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Riedl, Marc
Sent: Thursday, May 03, 2012 12:51 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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