[CIS PIDD] [cis-pidd] Low TRECs, nromal mitogens, progressive lymphopenia

Keller, Michael MKeller at childrensnational.org
Wed Jan 7 08:15:46 EST 2015


Hi Richard,

I agree with the previous posts that a hypomorphic SCID or related mutation would be a consideration, though completely normal mitogens would argue against that to a degree.   Is uric acid normal?

I think our approach would be to go ahead with sequencing, and check antibody and antigen responses in the next few months.

Best regards,
Mike


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

Michael D. Keller MD

Assistant Professor, Division of Allergy / Immunology

Center for Cancer and Immunology Research

Children's National Health System

111 Michigan Ave NW, Room 1W-314B

Washington, DC 20010

Clinic: 202.476.3016

Office: 202.476.5843

Fax: 202.476.2280

www.childrensnational.org


________________________________
From: Daniel Conway [dhconway at hotmail.com]
Sent: Tuesday, January 06, 2015 10:15 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Low TRECs, nromal mitogens, progressive lymphopenia

I am interested in another downstream function of genes involved in recombination- like specific antibody formation.

Sincerely,
Daniel H. Conway, MD
Assistant Professor of Pediatrics
St. Christopher's Hospital for Children
Drexel University College of Medicine

On Jan 6, 2015, at 9:37 PM, Richard Wasserman <drrichwasserman at gmail.com<mailto:drrichwasserman at gmail.com>> wrote:

IgM at three weeks of age was 46mg/dL

On Tue, Jan 6, 2015 at 8:21 PM, Cooper, Megan <Cooper_M at kids.wustl.edu<mailto:Cooper_M at kids.wustl.edu>> wrote:
Hi Richard - it looks as if all the populations are dropping, I might consider ADA (somewhat delayed onset).

Megan

Megan A. Cooper, MD, PhD
Assistant Professor, Department of Pediatrics
Division of Rheumatology
Washington University School of Medicine
Cooper_m at kids.wustl.edu<mailto:Cooper_m at kids.wustl.edu>
Lab website: http://research.peds.wustl.edu/Default.aspx?alias=research.peds.wustl.edu/Labs/Cooper_M<https://urldefense.proofpoint.com/v2/url?u=http-3A__research.peds.wustl.edu_Default.aspx-3Falias-3Dresearch.peds.wustl.edu_Labs_Cooper-5FM&d=AwMGaQ&c=Zoipt4Nmcnjorr_6TBHi1A&r=mERX_I8PKb0Uil9coedoT1CtvFqkSey45L0vbcX0oKI&m=XDGSt8E3aO2CwaJ0M_VDcJls09j3vHlFlqcXCgBuKWw&s=WuAW1f8At-utJgbqJWLIYYIUUF-_wFfDUY_0W-FkBQU&e=>

On Jan 6, 2015, at 7:47 PM, Richard Wasserman <drrichwasserman at gmail.com<mailto:drrichwasserman at gmail.com>> wrote:


Please give me your thoughts on the best next steps for this six month old male who is thriving and has never been infected.

July 3, 2014 - Date of Birth

July 4, 2014, July 11, 2014 - Very Low TRECs

July 25, 2014

Absolute Lymphocytes L 3428 (cells/uL) 3400-7600

Absolute Cd3+ Cells L 1845 (cells/uL) 2500-5500

Absolute Cd4+ Cells L 1435 (cells/uL) 1600-4000

Absolute Cd8+ Cells L 389 (cells/uL) 680-1700

Absolute Nk Cells (cd16+cd56+cells) 735 (cells/uL) 170-1100

Absolute Cd19+ Cells L 828 (cells/uL) 300-2000

Absolute Cd3+Cd4+Cd45RA+Cd62L+ 1260 (cells/uL) 1200-3600

Absolute Cd3+Cd4+Cd45RO+ 232 (cells/uL) 60-900

Absolute Cd3+Cd8+Cd45RA+Cd62L+ 363 (cells/uL) 3800-1300

Absolute Cd3+Cd4+Cd45RO+ 18 (cells/uL) 30-330

PHA, PWM and ConA stimulation was normal

January 5, 2015

Absolute Lymphocytes L 1559 (cells/uL) 4000-13500

Absolute Cd3+ Cells L 754 (cells/uL) 2160-5540

Absolute Cd4+ Cells L 547 (cells/uL) 1390-4080

Absolute Cd8+ Cells L 222 (cells/uL) 600-1490

Absolute Nk Cells (cd16+cd56+cells) 184 (cells/uL) 160-930

Absolute Cd19+ Cells L 519 (cells/uL) 740-2560

 Thanks,
Richard
--
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
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Fax (972) 566-8837<tel:%28972%29%20566-8837>
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--
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
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Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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