Table 3.
Profiles of survived and died FHL patients.
| Survived (n = 29) | Died (n = 14) | p-value | |
|---|---|---|---|
| Males/females | 13:16 | 10:4 | ns |
| Age at onset (mean, range) | 0.6 (0.1–12 years) | 0.3 (0.1–0.8 years) | ns |
| FHL2:FHL3 | 7:5 | 5:3 | ns |
| Manifestation at diagnosis (%) | |||
| Fever | 96 (27/28) | 93 (13/14) | ns |
| Hepatosplenomegaly | 82 (23/25) | 93 (13/14) | ns |
| Lymphadenopathy | 29 (8/28) | 7 (1/14) | ns |
| Skin eruption | 21 (6/28) | 0 (0/14) | ns |
| CNS abnormality | 21 (6/28) | 21 (3/14) | ns |
| Respiratory failure | 14 (4/28) | 14 (2/14) | ns |
| DIC | 25 (7/28) | 50 (7/14) | ns |
| CNS signs at HSCT (%) | 68 (19/28) | 64 (9/14) | ns |
| CSF pleocytosis | 44 (7/16) | 0 (0/6) | ns |
| MRI abnormality | 52 (13/25) | 50 (7/14) | ns |
| Convulsion | 37 (10/27) | 80 (7/14) | ns |
| Disturbed consciousness | 26 (7/27) | 21 (3/14) | ns |
| HSCT | |||
| Age at HSCT (mean, range) | 1.7 (0.4–15 years) | 1.1 (0.7–4.8 years) | ns |
| UGBT:others | 11:6 | 6:7 | ns |
| MAC:RIC | 20:7 | 9:5 | ns |
CNS, central nerves system; HSCT, hematopoietic stem cell transplantation; CSF, cerebrospinal fluid; UCBT, unrelated cord blood transplantation; MAC, myeloabrative conditioning; RIC, reduced-intensity conditioning; ns, not significant.