[CIS PIDD] [cis-pidd] Lymphopenia after thoracic duct ligation

Boyce, Thomas G., M.D. Boyce.Thomas at mayo.edu
Thu Jun 13 11:11:38 EDT 2013

Lisa,

By "thymic defect" do you mean DGS or thymectomy post cardiac surgery or either?

Tom


Thomas G. Boyce, MD, MPH
Pediatric Infectious Diseases and Immunology
Mayo Clinic
email: boyce.thomas at mayo.edu
phone: 507-255-8464
fax: 507-255-7767


From: bounce-43476309-183824398 at lists.clinimmsoc.org [mailto:bounce-43476309-183824398 at lists.clinimmsoc.org] On Behalf Of Kobrynski, Lisa
Sent: Thursday, June 13, 2013 8:57 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] Lymphopenia after thoracic duct ligation


We frequently see generalized lymphopenia (B and T cells) post cardiac surgery in our CICU (even in those who do not have 22q11DS/DGS or a chylothorax). The ones that do have a chylothorax often have profound, although temporary lymphopenia. Most of our babies that have a thymic defect have a preferential loss of T cells (so the B or NK cell % is increased). This is similar to the flow results for your patient. With lymphopenia due to chylothorax or lymphangiectasia alone the proportions are not usually so skewed. The absolute numbers might still be low, but with global losses the proportions are relatively preserved. I know that Michigan reported a couple of babies with low TREC on repeat NBS due to a chylothorax. They presented a poster at AAAI and I can't recall what their flow data looked like.



Lisa


Lisa Kobrynski, MD, MPH
Associate Professor of Pediatrics
Marcus Professor of Immunology
Section, Allergy/Immunology
________________________________
From: Soheil Chegini [schegini at yahoo.com]
Sent: Wednesday, June 12, 2013 10:44 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Lymphopenia after thoracic duct ligation
Another concern with thoracic duct ligation could be secondary intestinal lymphangiectasia, which would result in intestinal loss of lymphocytes that preferentially affects T cells and primarily CD4 cells. Diarrhea may not be distinguished as such at this age, and hypoproteinemia may be incorrectly attributed to his recent cardiothoracic surgery and nutritional issues or masked by IV replacement. Obviously, this cannot account for his abnormal TREC, but if a simple screening test identifies elevated alpha-1 antitrypsin in his stool that corroborates this assumption and can explain the observed decline in his counts, I would suggest looking further into this possibility.

Regards,
Soheil

From: Richard Wasserman <drrichwasserman at gmail.com>
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Wednesday, June 12, 2013 12:23 PM
Subject: [cis-pidd] Lymphopenia after thoracic duct ligation

Colleagues,
Two month old male with abnormal newborn TREC screen. Newborn screen on day of life 2 was normal. On day of life 4, presented with an interrupted aortic arch, AP window and ASD. Initial CBC showed a white count of 16,700, ALC 4800. IAA repair on day 8. Post-op chylothorax required chemical pleurodesis on DOL 30 but chylous drainage persisted and thoracic duct ligation was performed on DOL 41. From DOL 9 - 41 ALC ranged from 574 to 1782.
Repeat newborn screen showed very low TREC on DOL 16. On discharge at DOL 51, the patient's ALC was 3332. Follow up CBC on DOL 58 showed an ALC of 8692/mm3. Additionally, chromosomal microarray and FISH for DiGeorge Syndrome were normal.
There have been no significant infections There is no family history suggestive of immunodeficiency.
The abnormal TREC was felt to be related to the lymphocytopenia stemming from his chylothorax. We saw him at 63 days of life and appeared in good heath. The third newborn screen is pending. Studies are shown. Mitogen stimulation is pending.
T&B Lymphocyte/Nat Killer (T cell subsets)
Abs.CD19+ Lymphs H 3845 (/uL) 600-1900
% CD19+ Lymphs H 69.9 (%) 4.0-26.0
Absolute CD 3 L 787 (/uL) 2300-7000
% CD 3 Pos. Lymph. L 14.3 (%) 60.0-85.0
Absolute CD 4 Helper L 561 (/uL) 1700-5300
% CD 4 Pos. Lymph. L 10.2 (%) 41.0-68.0
Abs. CD 8 Suppressor L 226 (/uL) 400-1700
% CD 8 Pos. Lymph. L 4.1 (%) 9.0-23.0
CD4/CD8 Ratio 2.49 0.92-3.72
Ab NK (CD56/16) 726 (/uL) 200-1400
% NK (CD56/16) 13.2 (%) 3.0-23.0

WBC 10.9 (x10E3/uL) 5.0-12.4
RBC 3.75 (x10E6/uL) 2.72-4.84
Hemoglobin 10.3 (g/dL) 8.8-14.3
Hematocrit 31.0 (%) 26.6-41.0
MCV 83 (fL) 81-97
MCH 27.5 (pg) 27.1-34.0
MCHC 33.2 (g/dL) 31.9-36.0
RDW 16.0 (%) 12.2-16.4
Platelets 519 (x10E3/uL) 150-579
Neutrophils 23 (%) 10-42
Lymphs 51 (%) 3-11
Eos H 7 (%) 0-5
Basos 0 (%) 0-2
Neutrophils (Absolute) 2.5 (x10E3/uL) 0.6-4.4
Lymphs (Absolute) 5.5 (x10E3/uL) 1.4-8.6
Monocytes(Absolute) H 2.1 (x10E3/uL) 0.2-1.1
Eos (Absolute) H 0.8 (x10E3/uL) 0.0-0.4
Immunoglobulin M, Qn, Serum 24 (mg/dL) 0-145
Do you think we can account for the T cell lymphopenia and B cell lymphocytosis by the duct ligation alone?

Thanks,
--
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
---
The CIS-PIDD listserv is supported by:
[Image removed by sender.]
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org<https://cis.execinc.com/edibo/Signup> to join!
You are currently subscribed to cis-pidd as: schegini at yahoo.com<mailto:schegini at yahoo.com>.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824417.b1e54082bbbf6f760251291ea6ac37ed&n=T&l=cis-pidd&o=43472762


---

The CIS-PIDD listserv is supported by:

[Image removed by sender.]
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org<https://cis.execinc.com/edibo/Signup> to join!

You are currently subscribed to cis-pidd as: lkobryn at emory.edu<mailto:lkobryn at emory.edu>.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824542.3201f5eed0ab3c3c73bfa5803d66dafd&n=T&l=cis-pidd&o=43474702

________________________________

This e-mail message (including any attachments) is for the sole use of
the intended recipient(s) and may contain confidential and privileged
information. If the reader of this message is not the intended
recipient, you are hereby notified that any dissemination, distribution
or copying of this message (including any attachments) is strictly
prohibited.

If you have received this message in error, please contact
the sender by reply e-mail message and destroy all copies of the
original message (including attachments).

---

The CIS-PIDD listserv is supported by:

[Image removed by sender.]
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org<https://cis.execinc.com/edibo/Signup> to join!

You are currently subscribed to cis-pidd as: boyce.thomas at mayo.edu<mailto:boyce.thomas at mayo.edu>.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824398.f2b8412bd8ff7bcbcdffccce20d02a5f&n=T&l=cis-pidd&o=43476309

---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=43476610
or send a blank email to leave-43476610-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20130613/72ce5415/attachment-0001.html>
-------------- next part --------------
A non-text attachment was scrubbed...
Name: not available
Type: image/jpeg
Size: 620 bytes
Desc: image001.jpg
Url : <http://seven.pairlist.net/pipermail/pagid/attachments/20130613/72ce5415/attachment-0001.jpe>

More information about the PAGID mailing list