Characteristics of 14 CGD patients with IMI within 2 years prior to HSCT
| . | N = 14 (%) . |
|---|---|
| Sex | |
| Male | 13 (93) |
| CGD characteristics | |
| Median age at diagnosis of CGD (years) [IQR] | 1 [0.3–3] |
| Genetic pattern | |
| X-linked CGD | 13 (93) |
| AR | 1 (7) |
| History of inflammatory diseases | 5 (36) |
| Immunosuppressive treatmenta | 6 (43) |
| IMI characteristics | |
| Median age at diagnosis of IMI (years) [IQR] | 15 [10–27] |
| Infection status at the time of HSCT | |
| CR | 4 (28.5) |
| PR | 4 (28.5) |
| SR | 1 (7) |
| DP | 5 (36) |
| Median time between IMI and HSCT (months) [IQR] | 10 [6–21] |
| Median time between IMI and HSCT among patients with partial or stable responses and disease progression (months) [IQR] | 7 [4–17] |
| Fungal species | |
| Aspergillus spp | 10 (71) |
| Mucorales | 2 (14) |
| L. prolificans | 1 (7.5) |
| S. apiospermum | 1 (7.5) |
| Site of infection | |
| Lung only | 7 (50) |
| Brain only | 1 (7) |
| Disseminated infection | 6 (43) |
| HSCT | |
| Age at HCST (years) | 15 [10–30] |
| Median year of HSCT | 2018 [2015–2019] |
| Donor type | |
| MSD | 6 |
| MUD | 3 |
| MMUD | 4 |
| MMRD | 1 |
| Stem cell source | |
| BM | 10 (71) |
| PBSC | 4 (29) |
| CD34 cells (cells per kg bodyweight) [IQR] | 5.9 × 106 [4–9.2] |
| Conditioning intensity | |
| RTC | 1 (7) |
| RIC | 13 (93) |
| Engraftment | 12 (86) |
| Median time at neutrophil engraftment (days) [IQR] | 17 [15–22] |
| Median time of platelet recovery (days) [IQR] | 18 [13–21] |
| Medial duration of post-HSCT antifungal treatment (months) | 4 [3–11] |
| Outcome | |
| 1-year OS | 12 (86) |
| Median DHR at 1 mo after HSCT (%) | 89 [74–95] |
| 3-mo median chimerism status (% donor) | 98 [94–99] |
| 3-mo clinical and radiological response b | 8 (80) |
| 1-year persistent clinical and radiological cure | 12 (86) |
| . | N = 14 (%) . |
|---|---|
| Sex | |
| Male | 13 (93) |
| CGD characteristics | |
| Median age at diagnosis of CGD (years) [IQR] | 1 [0.3–3] |
| Genetic pattern | |
| X-linked CGD | 13 (93) |
| AR | 1 (7) |
| History of inflammatory diseases | 5 (36) |
| Immunosuppressive treatmenta | 6 (43) |
| IMI characteristics | |
| Median age at diagnosis of IMI (years) [IQR] | 15 [10–27] |
| Infection status at the time of HSCT | |
| CR | 4 (28.5) |
| PR | 4 (28.5) |
| SR | 1 (7) |
| DP | 5 (36) |
| Median time between IMI and HSCT (months) [IQR] | 10 [6–21] |
| Median time between IMI and HSCT among patients with partial or stable responses and disease progression (months) [IQR] | 7 [4–17] |
| Fungal species | |
| Aspergillus spp | 10 (71) |
| Mucorales | 2 (14) |
| L. prolificans | 1 (7.5) |
| S. apiospermum | 1 (7.5) |
| Site of infection | |
| Lung only | 7 (50) |
| Brain only | 1 (7) |
| Disseminated infection | 6 (43) |
| HSCT | |
| Age at HCST (years) | 15 [10–30] |
| Median year of HSCT | 2018 [2015–2019] |
| Donor type | |
| MSD | 6 |
| MUD | 3 |
| MMUD | 4 |
| MMRD | 1 |
| Stem cell source | |
| BM | 10 (71) |
| PBSC | 4 (29) |
| CD34 cells (cells per kg bodyweight) [IQR] | 5.9 × 106 [4–9.2] |
| Conditioning intensity | |
| RTC | 1 (7) |
| RIC | 13 (93) |
| Engraftment | 12 (86) |
| Median time at neutrophil engraftment (days) [IQR] | 17 [15–22] |
| Median time of platelet recovery (days) [IQR] | 18 [13–21] |
| Medial duration of post-HSCT antifungal treatment (months) | 4 [3–11] |
| Outcome | |
| 1-year OS | 12 (86) |
| Median DHR at 1 mo after HSCT (%) | 89 [74–95] |
| 3-mo median chimerism status (% donor) | 98 [94–99] |
| 3-mo clinical and radiological response b | 8 (80) |
| 1-year persistent clinical and radiological cure | 12 (86) |