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

Nacho Gonzalez nachgonzalez at gmail.com
Wed Jun 12 12:38:05 EDT 2013


Agree with Dr. Church,
Our cardiac surgeons "always" remove the thymus after sternotomy. They say
that surgery is easier for them. Here in Madrid we are a referral centre
for congenital cardiac surgery and we follow these thymectomized patients,
so I am pretty sure that they still remove the thymus completely

Regards,

Luis




2013/6/12 Church, Joseph <JChurch at chla.usc.edu>


> If the baby had open heart surgery for IAA it is likely that a

> thymectomy was done by the surgeons. This will result in persistently low

> TREC.****

>

> ** **

>

> J Church****

>

> Children’s Hospital Los Angeles****

>

> ** **

>

> *From:* Richard Wasserman [mailto:drrichwasserman at gmail.com]

> *Sent:* Wednesday, June 12, 2013 9:23 AM

>

> *To:* CIS-PIDD

> *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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