[CIS PIDD] [cis-pidd] cis-pidd digest: September 05, 2013

[Puck, Jennifer]

Dear Richard,
I agree we would evaluate for 22q deletion, which I do these days with a
copy number array that at my med center is quicker, cheaper and much more
informative than FISH since it reveals insertions and deletions throughout
the entire genome. Clinical findings can be subtle to nil, but the
implications for further management are huge.
I imagine by now that there would be naïve T cells, given their increasing
numbers, but would still get naïve/memory markers.
In CA we do not give these babies rotavirus vaccine. We would re-examine
them and monitor their specific antibody responses after the primary
series of 3 killed vaccines (at around 7 months), also re-checking
lymphocyte subsets at that time. When T cells exceed 1500 and all else is
well, we don't follow further.
Jennifer
-- 
Jennifer Puck, MD
Professor of Pediatrics
UCSF Box 0519
513 Parnassus Ave, HSE 301A
San Francisco, CA 94143-0519

email: puckj at peds.ucsf.edu
phone: 415 476-3181
FAX:   415 502-5127



On 9/6/13 8:00 PM, "CIS-PIDD digest" <cis-pidd at lists.clinimmsoc.org> wrote:


>CIS-PIDD Digest for Thursday, September 05, 2013.

>

>1. Positive NB Screen

>2. RE: Positive NB Screen

>3. Re: Positive NB Screen

>4. Re: Positive NB Screen

>5. Re: Positive NB Screen

>

>----------------------------------------------------------------------

>

>Subject: Positive NB Screen

>From: Richard Wasserman <drrichwasserman at gmail.com>

>Date: Fri, 6 Sep 2013 14:20:25 -0500

>X-Message-Number: 1

>

>I have been evaluating a normal term female with an abnormal SCID screen

>of

>"very low TRECs". The available data are below:

>

> Day of life 38

>

>Day of life 62

>

>Abs.CD19+ Lymphs L 544 (/uL) 600-1900 01

>

>Absolute CD 3 L 718 (/uL) 2300-7000 01

>

>Absolute CD 4 Helper L 538 (/uL) 1700-5300 01

>

>Abs. CD 8 Suppressor L 178 (/uL) 400-1700 01

>

>Ab NK (CD56/16) 482 (/uL) 200-1400 01

>

>WBC L 3.9 (x10E3/uL) 5.0-12.4 01

>

>Abs.CD19+ Lymphs 1059 (/uL) 600-1900 01

>

>Absolute CD 3 L 957 (/uL) 2300-7000 01

>

>Absolute CD 4 Helper L 682 (/uL) 1700-5300 01

>

>Abs. CD 8 Suppressor L 267 (/uL) 400-1700 01

>

>Ab NK (CD56/16) 795 (/uL) 200-1400 01

>

>WBC 5.1 (x10E3/uL) 5.0-12.4

>

>Day of life 25 PHA = 47%, IgM = 10ml/dL

>

>PHA day of life 47 = 111%

>

>Adenosine deaminase 15.02

>

>

>

>She has been well. As you can see, her PHA normalized and her counts are

>improving. I'd appreciate suggestions for further evaluation.

>

>Richard Wasserman

>Dallas

>-- 

>Richard L. Wasserman, MD, PhD

>DallasAllergyImmunology

>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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