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

[Soheil Chegini]

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  
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