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

[Sullivan, Kathleen]

I have seen very impressive immune deficiency after the high risk ALL chemo.  In two cases (an old protocol no longer in use) the immunologic recovery never occurred even at one year out.
On Jan 24, 2014, at 12:01 AM, Issekutz, Thomas wrote:


> 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

>  

>  

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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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