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

[Sullivan, Kathleen]

Yes- the chylous draining can definitely account for the findings but if it was IAA type B, definitely should check for chromosome 22q11.2 deletion.

Kate
On Jun 12, 2013, at 12:23 PM, Richard Wasserman wrote:


> 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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> 


Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363



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