[PAGID] Calcinosis cutis in an transplanted ADA-deficient child

[Luigi Notarangelo]

In Brescia, Italy (where I was before moving to Boston), we are following a
3-year-old female child with ADA deficiency, who has received HLA-identical
stem cell transplantation. At nearly 3 years fater transplant, she has
excellent T cell reconstitution (normal T numbers and function, 98%
donor-derived T cells), mixed B cell chimerism, normal immunoglobulin
production. Levels of toxic metabolites have dramatically dropped after
transplant. However, the child has developed calcinosi cutis, with multiple
lesions at soft tissues. There are no skeletal abnormalities. Levels of
calcium and of PTH are normal. Has anybody observed anything similar in the
context of severe PIDs or after stem cell transplant?


Luigi D. Notarangelo
Division of Immunology, Children's Hospital
Karp Family Research Building, 9th floor, Rm 09210
Boston, MA 02115
tel: 617-919-2276
FAX: 617-730-0709