[CIS PIDD] [cis-pidd] ADA SCID tx

[Cowan, Mort]

Dear Elena,

If indeed he has rejected the graft (and it may take a little time to determine that), you might consider PEG-ADA therapy to at least stabilize him and give him some time to recover from the initial exposure to chemotherapy?  Also, was the "HLA matched" CB allele matched and if so, 6/6 or 8/8??

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

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-----Original Message-----
From: Perez, Elena [mailto:e.perez13 at med.miami.edu] 
Sent: Wednesday, July 09, 2014 6:55 PM
To: CIS-PIDD
Subject: [cis-pidd] ADA SCID tx

Dear "transplant-for-Primary Immunodeficiency" community:

We have a 2mo boy with ADA deficiency picked up on NBS who received an HLA matched umbilical cord transplant on DOL45 after being conditioned with 50% dose reduced Busulphan, Fludarabine and ATG and rituximab, which was well tolerated. Stem Cell Dose: 16.7 x 10e5 CD34 pos cells/kg; 1.69 x 10e7 TNC/kg. 

Early evaluation of peripheral cell genotype unfortunately revealed mixed chimerism of 3% donor suggestive of primary graft failure. His most recent chimerism revealed 5% donor, and the transplanters are planning to send another one this week before deciding about retransplant. Today is +41d post transplant.

In anticipation of need for retransplant, I offered to post his case on the list serve for feedback to our transplanters. They are considering retransplant with umbilical cord blood but hesitant to redose chemo vs maternal haplo but clinimacs cell purification IND is not available here.  

He is infection free but has developed seizures that are controlled on Keppra. He is maintained on IVIG and all the usual prophylaxis and so far has remained free of detectable infections, except on presentation required oxygen which has improved to room air. 

He is on medicaid with very limited family resources, which makes it difficult to transfer care out of state.

Feedback from transplanters in the group appreciated, and will share with transplanters here.

thank you,
Elena Perez
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The CIS-PIDD listserv is supported by the Clinical Immunology Society
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