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

[John Ziegler]

Joe, It seems that despite the CD4 lymphopenia the child has good antibody production and doesn't need IVIG.

Cheers

John

From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Thursday, 23 July 2015 4:08 AM
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



---------------------------------------------------------------------
CONFIDENTIALITY NOTICE: This e-mail message, including any attachments,
is for the sole use of the intended recipient(s) and may contain confidential
or legally privileged information. Any unauthorized review, use, disclosure
or distribution is prohibited. If you are not the intended recipient, please
contact the sender by reply e-mail and destroy all copies of this original message.

---------------------------------------------------------------------

---

You are currently subscribed to cis-pidd as: j.ziegler at unsw.edu.au<mailto:j.ziegler at unsw.edu.au>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396640.adcca036b6460aa865f1c3c405d26562&n=T&l=cis-pidd&o=3000839

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3000839-96396640.adcca036b6460aa865f1c3c405d26562 at lyris.dundee.net<mailto:leave-3000839-96396640.adcca036b6460aa865f1c3c405d26562 at lyris.dundee.net>

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=3001718
or send a blank email to leave-3001718-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20150723/8a1290ab/attachment-0001.html>