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

Wed Jul 22 22:14:11 EDT 2015

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-------- Original message --------
From: "Boyce, Thomas G., M.D."
Date:07/22/2015 5:18 PM (GMT-05:00)
To: CIS-PIDD
Subject: RE: [cis-pidd] Baby with intestingal lymphangiectasia

This patient would qualify for PCP prophylaxis since his CD4 percentage is <15%.  In a child between 1 and 5 years of age, such as this patient, that corresponds to a CD4 absolute count <500 (which he also has).

From: Pere Soler Palacin [mailto:psoler at vhebron.net]
Sent: Wednesday, July 22, 2015 4:24 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Baby with intestingal lymphangiectasia

Dear all, in our experience, PLE (both post-Fontan and primary lymphangiectasia) remain free of infections and vaccine response is usually normal despite very low IgG levels. However, we have decided to put them all on IV or SC Ig therapy since no clear clinical evidence on how to manage this patients is available as far as I know and cases with serious infectious complications have been described. We only start cotrimoxazole when CD4+ count is below 250/mm3 or 15% depending on patients' age.
In my opinion, I'd not worry about IgA values since the patient is only 18 mo of age. Since lymphedema is described in GATA-2 deficient patients I would check his monocyte count.
Besides, would you consider putting all our data on PLE patients from an immunological point of view to shed some light on that field?

Hope this helps.

Best,

Pere

Pere Soler Palacín, MD, PhD.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron
Assistant Professor. Universitat Autònoma de Barcelona (UAB)
Pg. de la Vall d'Hebron, 119-129
08035 Barcelona. Spain.
Tel. 0034934893140  /  Fax 0034934893039

psoler at vhebron.net<mailto:psoler at vhebron.net>  /  34660psp at comb.cat<mailto:34660psp at comb.cat>
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________________________________
De: "Jennifer Heimall" <heimallj at email.chop.edu<mailto:heimallj at email.chop.edu>>
Per: "CIS-PIDD" <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Enviats: Dimecres, 22 de Juliol 2015 22:07:11
Assumpte: RE: [cis-pidd] Baby with intestingal lymphangiectasia

In our cohort of post-Fontan PLE patients we have an median IgG level about 200, but the 95% range does dip under 100mg/dL.

In the setting of no infections, I would not start immunoglobulin replacement, but would monitor closely and have a low threshold to start if under 150mg/dL and becoming symptomatic with infections.

Agree that if going to use it would use SubQ

Jen

Jennifer Heimall, MD
The Children's Hospital of Philadelphia
Medical Director Day Medicine
Allergy/Immunology Attending Physician
3550 Market Street
3rd Floor
Philadelphia, PA 19104
215-590-2549 (p)
215-590-4529 (f)

________________________________
From: Joshi, Avni Y., M.D. <Joshi.Avni at mayo.edu<mailto:Joshi.Avni at mayo.edu>>
Sent: Wednesday, July 22, 2015 4:00 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] Baby with intestingal lymphangiectasia

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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