Clinical findings for each age group
| . | Childhood onset (age, <9 y) . | AA onset . | Elderly onset (age, >45 y) . | P, childhood vs AA . | P, AA vs elderly . | P, child vs elderly . | Test . |
|---|---|---|---|---|---|---|---|
| Total, n | 23 | 64 | 13 | ||||
| Male sex, n (%) | 18 (78) | 33 (52) | 2 (15) | .029 | .030 | <.001 | Fisher's exact test |
| EBV-infected cell type, n (%) | |||||||
| CD8− | 21 (91) | 39 (66) | 7 (54) | .026 | .526 | .016 | Fisher's exact test |
| CD8+ | 2 (9) | 20 (34) | 4 (46) | ||||
| ML diagnosis during clinical course | 1 (6) | 15 (25) | 6 (46) | .171 | .173 | .025 | Fisher's exact test |
| Symptoms and signs at diagnosis, n (%) | |||||||
| Fever | 20 (87) | 56 (88) | 9 (69) | .99 | .112 | .225 | Fisher's exact test |
| Lymphadenopathy | 9 (39) | 36 (56) | 3 (23) | .783 | .030 | .141 | Fisher's exact test |
| Hepatosplenomegaly | 11 (65) | 45 (74) | 8 (62) | .545 | .499 | .99 | Fisher's exact test |
| Vasculitis | 1 (6) | 5 (8) | 0 (0) | .99 | .579 | .99 | Fisher's exact test |
| Aneurysm | 4 (24) | 4 (7) | 0 (0) | .064 | .99 | .113 | Fisher's exact test |
| Uveitis | 0 (0) | 3 (5) | 1 (8) | .99 | .546 | .433 | Fisher's exact test |
| Heart disorder | 2 (12) | 6 (10) | 0 (0) | .99 | .583 | .492 | Fisher's exact test |
| Gastrointestinal symptom | 4 (24) | 2 (3) | 1 (8) | .019 | .445 | .355 | Fisher's exact test |
| Neurological disorder | 1 (6) | 5 (8) | 1 (8) | .99 | .99 | .99 | Fisher's exact test |
| HLH | 5 (29) | 14 (23) | 5 (38) | .750 | .298 | .705 | Fisher's exact test |
| HMB | 8 (35) | 16 (25) | 1 (8) | .274 | .191 | .219 | Fisher's exact test |
| HV | 3 (13) | 4 (6) | 2 (15) | ||||
| WBC count, median (range) | 4200 (2100-5200) | 3900 (2450-5050) | 3 460 (3 100-4 400) | .685 | .725 | .357 | Mann-Whitney U test |
| Neutrophil count, median (range) | 1455 (1092-2096) | 2136 (1364-3096) | 2 110.6 (1 336-2 869) | .069 | .855 | .329 | Mann-Whitney U test |
| Hb, g/dL | 10.5 ± 1.5 | 11.7 ± 2 | 10.3 ± 2.3 | .030 | .033 | .767 | Student t test |
| Platelets, median (range), ×104/µL | 20 (14-24) | 14.8 (8.7-19.2) | 13.5 (3.6-17.7) | .078 | .257 | .018 | Mann-Whitney U test |
| ALT, median (range), U/L | 135 (28-443) | 59 (29-134) | 57 (28-117) | .131 | .836 | .163 | Mann-Whitney U test |
| sIL-2R, median (range), U/mL | 1655 (1186-2141) | 1242 (796-2302) | 1 097 (452-11 398) | .319 | .975 | .696 | Mann-Whitney U test |
| . | Childhood onset (age, <9 y) . | AA onset . | Elderly onset (age, >45 y) . | P, childhood vs AA . | P, AA vs elderly . | P, child vs elderly . | Test . |
|---|---|---|---|---|---|---|---|
| Total, n | 23 | 64 | 13 | ||||
| Male sex, n (%) | 18 (78) | 33 (52) | 2 (15) | .029 | .030 | <.001 | Fisher's exact test |
| EBV-infected cell type, n (%) | |||||||
| CD8− | 21 (91) | 39 (66) | 7 (54) | .026 | .526 | .016 | Fisher's exact test |
| CD8+ | 2 (9) | 20 (34) | 4 (46) | ||||
| ML diagnosis during clinical course | 1 (6) | 15 (25) | 6 (46) | .171 | .173 | .025 | Fisher's exact test |
| Symptoms and signs at diagnosis, n (%) | |||||||
| Fever | 20 (87) | 56 (88) | 9 (69) | .99 | .112 | .225 | Fisher's exact test |
| Lymphadenopathy | 9 (39) | 36 (56) | 3 (23) | .783 | .030 | .141 | Fisher's exact test |
| Hepatosplenomegaly | 11 (65) | 45 (74) | 8 (62) | .545 | .499 | .99 | Fisher's exact test |
| Vasculitis | 1 (6) | 5 (8) | 0 (0) | .99 | .579 | .99 | Fisher's exact test |
| Aneurysm | 4 (24) | 4 (7) | 0 (0) | .064 | .99 | .113 | Fisher's exact test |
| Uveitis | 0 (0) | 3 (5) | 1 (8) | .99 | .546 | .433 | Fisher's exact test |
| Heart disorder | 2 (12) | 6 (10) | 0 (0) | .99 | .583 | .492 | Fisher's exact test |
| Gastrointestinal symptom | 4 (24) | 2 (3) | 1 (8) | .019 | .445 | .355 | Fisher's exact test |
| Neurological disorder | 1 (6) | 5 (8) | 1 (8) | .99 | .99 | .99 | Fisher's exact test |
| HLH | 5 (29) | 14 (23) | 5 (38) | .750 | .298 | .705 | Fisher's exact test |
| HMB | 8 (35) | 16 (25) | 1 (8) | .274 | .191 | .219 | Fisher's exact test |
| HV | 3 (13) | 4 (6) | 2 (15) | ||||
| WBC count, median (range) | 4200 (2100-5200) | 3900 (2450-5050) | 3 460 (3 100-4 400) | .685 | .725 | .357 | Mann-Whitney U test |
| Neutrophil count, median (range) | 1455 (1092-2096) | 2136 (1364-3096) | 2 110.6 (1 336-2 869) | .069 | .855 | .329 | Mann-Whitney U test |
| Hb, g/dL | 10.5 ± 1.5 | 11.7 ± 2 | 10.3 ± 2.3 | .030 | .033 | .767 | Student t test |
| Platelets, median (range), ×104/µL | 20 (14-24) | 14.8 (8.7-19.2) | 13.5 (3.6-17.7) | .078 | .257 | .018 | Mann-Whitney U test |
| ALT, median (range), U/L | 135 (28-443) | 59 (29-134) | 57 (28-117) | .131 | .836 | .163 | Mann-Whitney U test |
| sIL-2R, median (range), U/mL | 1655 (1186-2141) | 1242 (796-2302) | 1 097 (452-11 398) | .319 | .975 | .696 | Mann-Whitney U test |
AA, adolescent/adult; ML, malignant lymphoma; Hb, hemoglobin; WBC white blood cell.