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

[Alan Redding]

Dear Dr. Grimbacher/all,

   Yes, I think, from a medico-legals standpoint, we should offer this
women immunoglobulin replacement, despite the fact that her infections
history has been relatively mild.  Now, from a purely medical
standpoint, do I need to perform a high-resolution CT scan on her, as many
of have suggested?  Or, if her PFT's are completely normal, can I just do
a chest X-ray?
   I have even read that patients with CVID should have
CT chests at regular intervals.  Is this plan better than ordering chest
X-rays and full PFT's at regular intervals?  Since CT chests deliver much
more radiation than chest X-rays, I do not want to irradiate anyone more
than necessary.
   I appreciate all of the wonderful advice that I have received from
around the world.

Sincerely,
 Alan

On Sun, May 6, 2012 at 5:54 AM, Grimbacher, Bodo <b.grimbacher at ucl.ac.uk>wrote:


> Dear all,

>

> What is the medico-legal situation with a patient with < 4g/L IgG in the

> US and such an infection profile?

>

> I was told of a court-trial in Hamburg in which the Hospital lost as they

> failed offering a your women IVIG treatment who went on developing

> debilitating bronchiectasis.

> So should we at least offer these type of patients IgG replacement?

>

> Prof. B. Grimbacher

> Director

> CCI - Centre of Chronic Immunodeficiencies

> Freiburg, Germany

>

> Am 03.05.12 18:44 schrieb "Alan Redding" unter <aredding99 at gmail.com>:

>

> >       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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