Patient and graft characteristics (median, ranges) in groups of patients receiving transplants from MMRDs and MUDs
Donors . | Patients . | Graft composition . | ||||
---|---|---|---|---|---|---|
HLA matching . | Age at HSCT, years . | Median FU, years . | Diagnosis . | NC, 108/kg . | CD34+, 106/kg . | TCRαβ+, 103/kg . |
MUD | ||||||
8/10 2 | 4.2 (0.54-17.6) | 2.9 (0.5-5.8) | WAS, 20 | 6.96 (2.82-22.17) | 10.99 (3.2-21.32) | 18 (0.8-461) |
9/10 16 | HLH, 13 | |||||
10/10 57 | CGD, 6 | |||||
CD40L, 5 | ||||||
IPEX, 3 | ||||||
SAP, 3 | ||||||
XIAP, 3 | ||||||
PIK3D, 3 | ||||||
SCN, 3 | ||||||
CHS, 2 | ||||||
WHIM, 1 | ||||||
STAT1, 1 | ||||||
CHH, 1 | ||||||
XLA, 1 | ||||||
РАMI, 1 | ||||||
undefined PID, 9 | ||||||
MMRD | ||||||
Mothers 16 | 2 (0.85-15.7) | 2.0 (1.2-4.9) | WAS, 16 | 8.52 (3.67-19.96) | 10.5 (6.3-14.9) | 13.97 (5-130) |
9/10 1 | SCN, 3 | |||||
8/10 1 | HLH, 2 | |||||
7/10 2 | XIAP, 1 | |||||
6/10 5 | undefined PID, 1 | |||||
5/10 7 | ||||||
Fathers 7 | ||||||
6/10 2 | ||||||
5/10 5 | ||||||
P value | .06 | .4 | .09 | .59 | .69 |
Donors . | Patients . | Graft composition . | ||||
---|---|---|---|---|---|---|
HLA matching . | Age at HSCT, years . | Median FU, years . | Diagnosis . | NC, 108/kg . | CD34+, 106/kg . | TCRαβ+, 103/kg . |
MUD | ||||||
8/10 2 | 4.2 (0.54-17.6) | 2.9 (0.5-5.8) | WAS, 20 | 6.96 (2.82-22.17) | 10.99 (3.2-21.32) | 18 (0.8-461) |
9/10 16 | HLH, 13 | |||||
10/10 57 | CGD, 6 | |||||
CD40L, 5 | ||||||
IPEX, 3 | ||||||
SAP, 3 | ||||||
XIAP, 3 | ||||||
PIK3D, 3 | ||||||
SCN, 3 | ||||||
CHS, 2 | ||||||
WHIM, 1 | ||||||
STAT1, 1 | ||||||
CHH, 1 | ||||||
XLA, 1 | ||||||
РАMI, 1 | ||||||
undefined PID, 9 | ||||||
MMRD | ||||||
Mothers 16 | 2 (0.85-15.7) | 2.0 (1.2-4.9) | WAS, 16 | 8.52 (3.67-19.96) | 10.5 (6.3-14.9) | 13.97 (5-130) |
9/10 1 | SCN, 3 | |||||
8/10 1 | HLH, 2 | |||||
7/10 2 | XIAP, 1 | |||||
6/10 5 | undefined PID, 1 | |||||
5/10 7 | ||||||
Fathers 7 | ||||||
6/10 2 | ||||||
5/10 5 | ||||||
P value | .06 | .4 | .09 | .59 | .69 |
CGD, chronic granulomatous disease; CHH, cartilage hair hypoplasia; CHS, Chediak–Higashi syndrome; CD40L, CD40L deficiency; HLH, hemophagocytic lymphohistiocytosis; IPEX, IPEX syndrome (FOXP3 deficiency); РАMI, PSTPIP1-associated myeloid-related proteinemia inflammatory syndrome; PIK3D, PIK3D defect; SAP, X-linked lymphoproliferative disease, type 1 (SAP deficiency); SCN, severe congenital neutropenia; STAT1, STAT1 defect; WHIM, warts, hypogammaglobulinemia, immunodeficiency and myelokathexis syndrome (CXCR4 defect); XIAP, X-linked lymphoproliferative disease, type 2 (XIAP deficiency); XLA, X-linked agammaglobulinemia (BTK deficiency).