[CIS PIDD] [cis-pidd] stopping Adagen prior to transplant

[CIS-PIDD]

Dear Richard,
in a case like that we would stop about 2-4 weeks prior to conditioning. But since the plan is to use fully myeloablative conditioning and use ATG, you may not need to stop it until the beginning of conditioningm, if you feel that he urgently needs it. I would very, very strongly suggest to use ATG or other serotherapy!
BR
Michael Albert


Von: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] Im Auftrag von CIS-PIDD
Gesendet: Mittwoch, 12. April 2017 17:35
An: CIS-PIDD
Betreff: [cis-pidd] stopping Adagen prior to transplant

Colleagues,
I need a recommendation on the timing of stopping Adagen prior a fully matched MUD in a 19 yo ADA deficiency SCID who was diagnosed at about 15 months of age when he presented with multi system organ failure following varicella vaccination. Except for an episode of cryptococcal meningitis at about age 12 he had done relatively well on Adagen, IG, TMP/SMX and, since the meningitis, fluconazole until 1.5 years ago when he presented with fever, weight loss and chronic diarrhea.

He was found to have a very significant peripheral and bone marrow NK lymphocytosis and was thought to have an indolent NK leukemia. He was treated with supportive care but continued to deteriorate and developed pancytopenia. Colonoscopy and repeat bone marrow nine months ago showed MAI. He has been treated with a multidrug regimen for the MAI and GI symptoms have resolved, NK lymphocytosis has improved but the pancytopenia has not. He is transfusion dependent and has shown no signs of marrow recovery despite adequate nutrition.

A well matched donor has been identified and the plan is to myeloablate with busulfan and cyclophosphamide. The transplanters are considering ATG as well. The question is when to stop Adagen?

Thanks,
Richard
--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
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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