[CIS PIDD] [cis-pidd] Baby with intestingal lymphangiectasia

Joshi, Avni Y., M.D. Joshi.Avni at mayo.edu
Wed Jul 22 16:00:10 EDT 2015 

Hi Joe,
Have you ruled out Emberger syndrome(?GATA2)?

Thanks,
Avni

Mayo Clinic

From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Wednesday, July 22, 2015 2:31 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] Baby with intestingal lymphangiectasia

Thanks, All.

IF I were to try Ig it would be via SC route.  But should I even try in a patient with no infections?

Richard:
I think the reason this kid has no IgA is because she is losing so much Ig via her gut.  An IgG of 83 is pretty darn low for a protein losing process.  We see a fair number of PLEs secondary to Fontan heart procedures, and they usually aren’t this low.

Our lab does not normalize mitogen preps for lymphocyte counts.  So, I’m not too worried about her T-cells.

Regarding ticks vs fleas vs both, we will continue to watch her carefully.

JC

From: Richard Wasserman [mailto:drrichwasserman at gmail.com]
Sent: Wednesday, July 22, 2015 12:16 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Baby with intestingal lymphangiectasia

Joe,
Why is there no IgA? That concerns me a little. Most of the PLE's do pretty well without supplementation. Does your lab normalize mitogen result for lymphocyte count? I'd hate to give her two problems but while most dogs have ticks OR fleas some have ticks AND fleas.
Richard Wasserman
Dallas

On Wed, Jul 22, 2015 at 1:28 PM, Nacho Gonzalez <nachgonzalez at gmail.com<mailto:nachgonzalez at gmail.com>> wrote:
Dear Dr. Church,

the last 2 XLA patients I have seen recently were under the first year of life. They had poor vascular access... every IVIG infusion was a pain. Now they are under SCIG and doing fine. But going back to your patient, I do not think she needs IgG replacement therapy if she is free of infections.

Best regards,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies Unit
Pediatric Hematology & Oncology Unit
Hospital 12 octubre
Madrid. Spain

2015-07-22 20:19 GMT+02:00 Verbsky, James <jverbsky at mcw.edu<mailto:jverbsky at mcw.edu>>:
Not sure if this will work as well in teliangiectasia but in other PLE states subQ has been beneficial…might be tough in an 18mo..but it’s a thought

james

From: Church, Joseph [mailto:JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>]
Sent: Wednesday, July 22, 2015 1:08 PM
To: CIS-PIDD
Subject: [cis-pidd] Baby with intestingal lymphangiectasia

Colleagues:

I have consulted on an 18 month-old girl with intestinal lymphangiectasia and unilateral lymphedema.  She has experienced no infectious complications.

However, labs demonstrate
            Serum albumin 2.3 g/dL
            IgG      83 mg/dL
            IgA      <7 mg/dL
            IgM      27 mg/dL
            Tetanus antibody 0.49 IU/mL (considered protective)
            Haemophilus influenzae antibody 0.48 mcg/mL (considered unprotective)
            Pneumococcal antibody response to Prevnar 13 positive (>1.3 mcg/ml) for 10 of 12 serotypes tested.
            CD3+ 46% (1399/mcL)
            CD4+ 14% (328/mcL)
            CD8+ 46% (1113/mcL)
            CD19+ 24% (590/mcL)
            NK 16% (391/mcL)
            PHA and PWM responses decreased (~ 25% of concurrent control)

The child is clearly losing protein, immunoglobulins and T-cells (CD4+ > CD8+), but has fair circulating antibody responses to tetanus toxoid and conjugated pneumococcal vaccine.

I would appreciate your opinions regarding immunoglobulin replacement therapy.

Joe Church, MD
Children’s Hospital Los Angeles



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--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
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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