[CIS PIDD] [cis-pidd] T cell def. post chemo

[Cowan, Mort]

Dear Thomas,

We had a very similar patient who had ALL ~1 year of age, treated aggressively and at ~2 yrs of age presented with laboratory findings c/w SCID (virtually absent CD3 numbers and function), on IVIG.  We went back to her newborn Guthrie card and she had normal TRECs and there was no evidence of PID in any of her pre-ALL blood test although there weren't many available.  We transplanted her with a 10/10 MUD bone marrow without any conditioning and she engrafted with 100% donor chimerism.  We didn't use any serotherapy and she did develop severe GVHD from which she recovered.

Mort

Morton J. Cowan, M.D.
Professor of Pediatrics
Chief, Allergy, Immunology, and Blood and Marrow Transplant Division
UCSF Children's Hospital, Room M659
505 Parnassus Ave
San Francisco, CA 94143-1278

Phone: 415-476-2188
FAX: 415-502-4867

**Confidentiality Notice** This email communication and any attachments may contain confidential and privileged information for the use of the designated recipients named above. Distribution, reproduction or any other use of this transmission by any party other than the intended recipient is prohibited.

From: Issekutz, Thomas [mailto:Thomas.Issekutz at iwk.nshealth.ca]
Sent: Thursday, January 23, 2014 9:02 PM
To: CIS-PIDD
Subject: [cis-pidd] T cell def. post chemo

Dear Colleagues,

I was consulted by our hematology service about a two and a half year old little girl who was being treated for Infantile ALL and would appreciate any advice.  This child is of aboriginal background in Nova Scotia.  She presented with anemia and thrombocytopenia at four months of age and a CD10- pre- B cell ALL. She went through four cycles of very intense chemotherapy over 22 months according to a COG protocol for this very aggressive ALL.  We were consulted because after the last cycle she did not seem to be recovering clinically.

She developed an outbreak of shingles that then became disseminated varicella. She had norovirus in her stools that would not clear, and C. difficile that required several courses of treatment to improve. She had repeated spiking fevers with negative cultures that only improved following amphotericin. She also developed pneumatosis intestinalis that has persisted over months.  Except for the varicella other herpes virus pcrs were all negative.

Immunologically, her IgG and IgM were normal at the time of presentation and throughout most of her chemotherapy.  However, after 18 months of chemo IgG was consistently low, but IgM was normal and IgA was mostly low. Her hemoglobin, platelet counts, ANC and ALC were all normal. Her ALC was 2000.  However, this consisted of 75% B cells and 0.5% CD3+CD4+, 0% CD3+CD8+, 23% NK cells. The few CD4s were only 9% CD45RA+ and 90% CD45RO+.  Thus virtually no CD4s. These measurement were done three and a half months after her last chemotherapy and again a month later without any change in CD4s. She is IVIG dependent. She also receives po IVIG for the norovirus with some clinical benefit re diarrhea.

Because the community she is from has had several children with SCID due to a mutation in RAG-2; she was checked for this and was normal (no heterozygosity even). Her first lymphocyte analysis at presentation and occasionally during follow up by hematology appeared to show normal numbers of T cells. Thus we think it is likely that she has had her T cells depleted and possibly her thymus severely injured by the intense chemo.  The possibility of a bone marrow transplant has been explored and a grandfather is a full 10 out of 10 match, because it is a fairly small community.

I would appreciate any thoughts on this patient. One other option that has also been considered is the use of IL-7 to stimulate T cell recovery as has been done in some cases post HSCT. Would this be worth trying, and does anyone have any experience with this. Should we just keep waiting to see if T cells return? It has now been five months since her last chemotherapy. The leukaemia is in remission at least for the moment. Also any previous experience in dealing with this scenario would be appreciated.

Sincerely

Thomas Issekutz MD
Professor and Head, Division of Immunology,
Department of Pediatrics
Dalhousie University/IWK Health Center
5850 University Avenue
Halifax, Nova Scotia B3K 6R8
CANADA
ph 902-470-8933
fax 902-470-7812



---

The CIS-PIDD listserv is supported by:

[http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org<mailto: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: mcowan at peds.ucsf.edu<mailto:mcowan at peds.ucsf.edu>.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824440.50c3c9deefef7b074fcf69d24f6b32e1&n=T&l=cis-pidd&o=44434620

---
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=44437078
or send a blank email to leave-44437078-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20140124/e53265b4/attachment.html>