[CIS PIDD] [cis-pidd] Newborn with Multiple Intestinal Atresia and Immune Deficiency
Yeşim Yılmaz Demirdağ
dryesimyilmaz at gmail.com
Fri Jul 12 18:58:58 EDT 2013
Dear all,
I have a very challenging case I would like to discuss:
12 day baby girl, ex-premie at 35 wks EGA.
Mother is an American/ Irish father is Ashkenazi Jewish/Irish. There is no
consanguinity. Patient is a product of second pregnancy through IVF, the
first pregnancy (again IVF) ended early due to unknown reasons.
Last week, patient underwent an extensive intestinal surgery including
gastoduodenostomy, small bowel resection x 7, cecectomy, appedectomy,
enteroenterostomy x 8, duodenostomy, sigmoid colostomy, mucus fistula x2,
and placement of gastrostomy tube.
Her findings on day 3 of life;
ALC: 1200 cells/microliter
AMC: 3200 cells/microliter
ANC: 6500 cells/microliter
CXR: no thymus shadow
day 4: IgA and IgM are undetectable, IgG: 561 mg/dl
Lymphocyte subsets: CD3 absolute: 350 cells/microliter (67%)
CD4 abs: 314 cells/microliter (60%)
CD8: 3 cells/microliter (3%)
CD19: 89 cells/microliter
CD16+CD56: 69 cells/microliter
Day 7 ALC is 1600 cells/microliter
Day 10 ALC: 700 cells/microliter
Day 12 (today) after spiking a temp of 100.2 her WBC went up to 22,000 and
ALC is now 2100.
Other labs:
% CD45RA 56 % 15-70
Absolute CD45RA 192 L /uL 200-3400
% CD45RO 15 % 5-30
Absolute CD45RO 52 /uL 50-1500
% CD2 87 % 55-88
Absolute CD2 381 L /uL 3800-5300
% HLA-DR 9 L % 11-45
Absolute HLA-DR 38 L /uL 430-3300
TREC: 539 copies per million CD3 cells (normal for age > 4168).
Newborn screening test is negative x 2.
Lymph proliferation: pending
TTC7A mutation assay: pending
My questions are:
Would you transplant this baby? Our BMT team told me that it is very
unlikely that they can find an unrelated mathched donor, and they would
like to use mother's BM.
What type of donor would you recommend if there is no UMD? Cord blood vs
mother's BM?
What type of conditioning would you do?
What would you recommend for the donor T cell depletion?
Thank you and have a great weekend!
Yeshim
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