Initial characteristics, treatment approach and outcome of 75 patients with LBCL-IRF4+
| Variable . | Number of patients . | % . |
|---|---|---|
| Sex | ||
| Male | 37 | 49.3 |
| Female | 38 | 50.7 |
| Age | ||
| Median (y) | 10.0 | - |
| Range (y) | 3.0-21.7 | - |
| <10 y | 37 | 49.3 |
| ≥10 y | 38 | 50.7 |
| Pre-existing disorder | ||
| Yes† | 4 | 5.3 |
| No | 70 | 93.3 |
| Serum LDH level | ||
| <2 × ULN | 65 | 86.7 |
| ≥2 × ULN | 1 | 1.3 |
| Not available | 9 | 12.0 |
| Stage of disease19,20 | ||
| I | 26 | 34.7 |
| II | 39 | 52.0 |
| III | 10 | 13.3 |
| IV | 0 | - |
| Site of disease | ||
| Head-and-neck | 57 | 76.0 |
| Pharynx§ | 43 | - |
| Lymph nodes only | 13 | - |
| Epiglottis | 1 | - |
| Abdomen | 14 | 18.7 |
| GIT | 13 | - |
| Other | 1 | - |
| Other sites|| | 4 | 5.3 |
| Resection status | ||
| Complete | 19 | 25.3 |
| Incomplete/biopsy | 55 | 73.3 |
| Not available | 1 | 1.3 |
| Treatment #1 | ||
| Risk groups∗ | ||
| Low and intermediate | 53 | 70.7 |
| High | 3 | 4.0 |
| Miscellaneous | 6 | 8.0 |
| Watchful waiting | 3 | 4.0 |
| Not available‡ | 10 | 13.3 |
| Treatment #2 | ||
| Chemotherapy | 62 | 82.7 |
| + radiotherapy | 2 | 3.2 |
| + rituximab | 14 | 22.6 |
| Watchful waiting | 3 | 4.0 |
| Not available‡ | 10 | 13.3 |
| Complete resection | ||
| Watchful waiting | 2 | 10.5 |
| Chemotherapy | 13 | 68.4 |
| + Radiotherapy | 0 | - |
| Not available | 4 | 21.1 |
| Outcome of 65 patients | ||
| Relapse | 1 | 1.3 |
| Second malignancy | 0 | - |
| Death | 2 | 2.7 |
| 2.5-y EFS | 96 ± 3% | - |
| 2.5-y OS | 96 ± 3% | - |
| Follow-up | ||
| Median (y) | 2.84 | - |
| Range (y) | 0.09-11.26 | - |
| Variable . | Number of patients . | % . |
|---|---|---|
| Sex | ||
| Male | 37 | 49.3 |
| Female | 38 | 50.7 |
| Age | ||
| Median (y) | 10.0 | - |
| Range (y) | 3.0-21.7 | - |
| <10 y | 37 | 49.3 |
| ≥10 y | 38 | 50.7 |
| Pre-existing disorder | ||
| Yes† | 4 | 5.3 |
| No | 70 | 93.3 |
| Serum LDH level | ||
| <2 × ULN | 65 | 86.7 |
| ≥2 × ULN | 1 | 1.3 |
| Not available | 9 | 12.0 |
| Stage of disease19,20 | ||
| I | 26 | 34.7 |
| II | 39 | 52.0 |
| III | 10 | 13.3 |
| IV | 0 | - |
| Site of disease | ||
| Head-and-neck | 57 | 76.0 |
| Pharynx§ | 43 | - |
| Lymph nodes only | 13 | - |
| Epiglottis | 1 | - |
| Abdomen | 14 | 18.7 |
| GIT | 13 | - |
| Other | 1 | - |
| Other sites|| | 4 | 5.3 |
| Resection status | ||
| Complete | 19 | 25.3 |
| Incomplete/biopsy | 55 | 73.3 |
| Not available | 1 | 1.3 |
| Treatment #1 | ||
| Risk groups∗ | ||
| Low and intermediate | 53 | 70.7 |
| High | 3 | 4.0 |
| Miscellaneous | 6 | 8.0 |
| Watchful waiting | 3 | 4.0 |
| Not available‡ | 10 | 13.3 |
| Treatment #2 | ||
| Chemotherapy | 62 | 82.7 |
| + radiotherapy | 2 | 3.2 |
| + rituximab | 14 | 22.6 |
| Watchful waiting | 3 | 4.0 |
| Not available‡ | 10 | 13.3 |
| Complete resection | ||
| Watchful waiting | 2 | 10.5 |
| Chemotherapy | 13 | 68.4 |
| + Radiotherapy | 0 | - |
| Not available | 4 | 21.1 |
| Outcome of 65 patients | ||
| Relapse | 1 | 1.3 |
| Second malignancy | 0 | - |
| Death | 2 | 2.7 |
| 2.5-y EFS | 96 ± 3% | - |
| 2.5-y OS | 96 ± 3% | - |
| Follow-up | ||
| Median (y) | 2.84 | - |
| Range (y) | 0.09-11.26 | - |
EFS, event-free survival; COP, cyclophosphamide, oncovin, prednisone; GIT, gastrointestinal tract; LDH, lactate dehydrogenase; OS, overall survival; R-CHOP, rituximab-cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone; R-CHOEP, rituximab-cyclophosphamide, hydroxydaunorubicin, oncovin, etoposide, prednisone; R-EPOCH, rituximab-etoposide, prednisone, oncovin, cyclophosphamide, hydroxydaunorubicin; ULN, upper limit of normal.
NHL-BFM therapy, n = 28, including 10 ‡ with no risk groups available due to inclusion in an ongoing trial; FAB/LMB therapy, n = 20; AIEOP LNH therapy, n = 6; SHOP LNH therapy, n = 5; JPSLG B-cell non-Hodgkin lymphoma therapy, n = 7; watchful waiting, n = 3; miscellaneous therapy, n = 6 (watchful waiting after only a prephase with COP, n = 1; R-CHOP, n = 2; R-EPOCH, n = 1; R-CHOEP, n = 1; details of chemotherapy not available, n = 1). Please refer to references 13 to 18.
Ataxia telangiectasia; Griscelli syndrome type-2; severe combined immunodeficiency: ZAP70 + FFAP24 mutations; selective IgA deficiency; notably, 1 patient with hypothyroidism was also reported, but not accounted for a predisposing disorder.
No risk group available due to inclusion in an ongoing trial.
Including 13 patients with additional involvement of the cervical lymph nodes.
Head-and-neck + abdomen, n = 1 (stage III); liver only, n = 1 (stage III); peripheral lymph nodes outside the head-and-neck region, n = 2: inguinal and pelvic lymph nodes (stage II), inguinal and mediastinal lymph nodes (stage III).