[CIS PIDD] [cis-pidd] Thymectomy and Hodgkin Disease

Sun Jun 25 14:20:54 EDT 2017

  Did she have a post-op chylothorax either with the original or subsequent cardiac procedures?  I think it is possible to deplete clones that are hard to replace with either a prolonged chylothorax or protein-losing enteropathy in someone who has had a complete or subtotal thymectomy.  Have you looked at her V beta repertoire?

  In the recent paper looking at lymphopenia in 178 patients who had had the Fontan procedure as a part of correction of congenital single ventricle, one patient died of EBV-associated Hodgkin's lymphoma (Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure... ; Morsheimer et al 2016).  The incidence of Hodgkin's lymphoma in older adolescents and young adults in one reference I found is about 32 per million per year.  I realize that you can't say much with such a small sample, but it seems the incidence might be increased among thymectomized patients.  The authors noted in the above paper that the proportion of patients with T cell lymphopenia increases with age.  Perhaps Kate could comment since she is a coauthor.

  A lot of patients with severe congenital heart disease with or without chromosome 22q11.2 deletions are entering adulthood now, and long-term studies could be helpful to see what the future may hold for them.  I think the day may come when rather than going into the wastebin, the patient will have an autotransplant to salvage their thymic tissue and preserve their T cell production throughout childhood and young adulthood.

Prescott

T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel: 205-638-6457
Fax: 205-975-7080
Cell: 205-999-7688

From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Sunday, June 25, 2017 11:08 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: RE: [cis-pidd] Thymectomy and Hodgkin Disease

Thanks Pere, Kate:

Microarray did not demonstrate copy number variant at 22q.  It did show a microduplication  at 1.21.1 which is associated with TOF.

In my brief review of the literature I did not see an association of 1.21.1 microdeletion with immune deficiency or lymphoma.

Joe Church

________________________________
From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [cis-pidd at lyris.dundee.net] on behalf of CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Sunday, June 25, 2017 6:57 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] Thymectomy and Hodgkin Disease (EXTERNAL EMAIL)
That's pretty low for post thymectomy T cells. She is not 22q?

Kate Sullivan
Sent from my iPhone

On Jun 24, 2017, at 3:52 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Colleagues:

We are seeing a 19 year-old young lady who was born with tetralogy of Fallot.  During surgical correction thymectomy is part of the procedure.  She had a long history of recurrent otitis and at 17yo immunologic evaluation demonstrated borderline IgG, marginal antibody to pneumococcus, normal antibody to tetanus toxoid and Hib.  T-cells were 60% (874), B-cells 11% (156), NK cells26% (383).  She was not treated with Ig.

Seven months later she was diagnosed with EBV-positive Hodgkin disease.  She is currently in remission.

My question:  "Does neonatal thymectomy increase the risk for Hodgkin disease?"

Joe Church

Children's Hospital Los Angeles

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