Table 1.

Patient and graft characteristics (median, ranges) in groups of patients receiving transplants from MMRDs and MUDs

DonorsPatientsGraft composition
HLA matchingAge at HSCT, yearsMedian FU, yearsDiagnosisNC, 108/kgCD34+, 106/kgTCRαβ+, 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 
DonorsPatientsGraft composition
HLA matchingAge at HSCT, yearsMedian FU, yearsDiagnosisNC, 108/kgCD34+, 106/kgTCRαβ+, 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).

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