Table 2.
| A. Primary cause of death between 2 – 6 years | ||||
|---|---|---|---|---|
| Causes of death | SCID N=10 |
Non-SCID N=12 |
IEM N=27 |
Total N=49 |
| Chronic GVHD | 2 | 5 | 7 | 14 |
| Infection without GVHD* | 3 | 2 | 6 | 11 |
| Organ failure** | 3 | — | 10 | 13 |
| PTLD – EBV positive | 1 | 1 | — | 2 |
| Acute myeloid leukemia | — | 2 | — | 2 |
| Graft failure | — | 1 | — | 1 |
| Seizure | — | — | 1 | 1 |
| Accidental death | — | 1 | 1 | 2 |
| Acute abdomen | — | — | 1 | 1 |
| Not reported | 1 | — | 1 | 2 |
| B. Primary cause of death beyond 6 years | ||||
|---|---|---|---|---|
| Causes of death | SCID N=4 |
Non-SCID N=3 |
IEM N=10 |
Total N=17 |
| Chronic GVHD | — | — | 2 | 2 |
| Infection without GVHD* | 2 | 1 | 2 | 5 |
| Organ failure** | 1 | — | 2 | 3 |
| Brain stem glioma | — | 1 | — | 1 |
| Not reported | 1 | 1 | 4 | 6 |
Abbreviations: GVHD = graft-versus-host disease; PTLD-EBV = post-transplant lymphoproliferative disease – Epstein-Barr virus associated
*
Infection without GVHD: bacterial (n=8), viral (n=1) and no isolate (n=2)
**
Organ failure: multi-organ failure (n=1 IEM), cardiac (n=2 SCID; n=3 IEM) and pulmonary (n=1 SCID; n=5 IEM)
Abbreviations: GVHD = graft-versus-host disease
*
Infection without GVHD: bacterial infection (n=3), viral (n=1) and no isolate (n=1)
**
Organ failure: cardiac failure (n=1 SCID), pulmonary failure (n=1 IEM) and progressive neurological deterioration, Gaucher disease (n=1)