Fisher, Becky Rion, & Others

December 12, 2005

Group,

Just to clarify, I was writing of X-linked SCID BMTs, not all PID BMTs, as I

only have experience with X-SCID. X-SCID kids have no working immune

system, and therefore require a BMT from a healthy person in order to live.

Non-X-SCID PID patients may have partially working immune systems which may

respond differently to BMTs. Off all the 13 or so different types of SCID,

X-linked SCID is the type least likely to obtain B cell function

post-transplant. The better the match between donor's and recipient's Human

Leukocyte Antigens (HLA), the more likely it is that the BMT will succeed

without major problems. Other PID patients have received BMTs and gained

both normal T and B cell function. Every patient is different, and may

react differently. There are no guarantees.

A bone marrow transplant (BMT) is the transplant of non-embryonic stem cells

from a healthy donor into a recipient. X-SCID kids (host), when given a

BMT, cannot reject the BMT (graft) because they originally have no working

immune system (defense). When Graft Versus Host Disease (GVHD) presents, it

is a good sign that the graft is beginning to " take " (work), and that the

graft recognizes its host is " foreign " . A little GVH is good, but too much

GVH can result in the body's depletion of the host's blood products that are

within the host's bone marrow.

For reasons too difficult to explain here, although the match was good, in

's case, the GVH was severe, and depleted his bone marrow of its blood

products, requiring frequent transfusions. The 2nd BMT saved his life, and

we saw his counts rise dramatically in 3-4 days. We saw no GVH from the 2nd

BMT.

Sorry if this issue has become as clear as mud! Good resources are

Antipov, mother to , 4 years old, X-SCID, 2 BMTS @ Duke, normal T

cell function, SCIG weekly

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