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

[CIS-PIDD]

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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