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

[Grimbacher, Bodo]

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

> 

> 

>     

>