Main characteristics of patients with FHL
. | Patients with FHL2, UPN . | . | . | . | . | Patients with FHL3, UPN . | . | . | . | . | . | . | . | |||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Characteristic . | 210 . | 235 . | 256 . | 314 . | 306 . | 180 . | 225 . | 237 . | 249 . | 277 . | 289 . | 293 . | 336 . | |||||||||||
Age at diagnosis, mo | 2 | 3 | 60 | 2 | 4 | 2 | 144 | 84 | 96 | 108 | 3 | 2 | 4 | |||||||||||
Sex | M | F | M | F | F | F | F | M | F | M | M | F | M | |||||||||||
Fever | + | + | + | + | + | + | + | + | + | + | + | - | + | |||||||||||
Splenomegaly | + | + | + | + | + | + | + | + | + | + | + | + | + | |||||||||||
Cytopenia | + | + | + | + | + | + | + | + | + | + | + | + | + | |||||||||||
Hypertriglyceridemia | ND | + | + | + | + | + | + | ND | - | + | - | + | + | |||||||||||
Hypofibrinogenemia | + | - | + | + | + | + | - | + | - | + | + | + | + | |||||||||||
HSCT | Y | Y | N | Y | Y | N | N | Y | Y | Y | N | Y | Y | |||||||||||
Outcome | DoD | Alive | Alive | DoD | Alive | DoD | DoT | Alive | Alive | Alive | DoD | Alive | Alive |
. | Patients with FHL2, UPN . | . | . | . | . | Patients with FHL3, UPN . | . | . | . | . | . | . | . | |||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Characteristic . | 210 . | 235 . | 256 . | 314 . | 306 . | 180 . | 225 . | 237 . | 249 . | 277 . | 289 . | 293 . | 336 . | |||||||||||
Age at diagnosis, mo | 2 | 3 | 60 | 2 | 4 | 2 | 144 | 84 | 96 | 108 | 3 | 2 | 4 | |||||||||||
Sex | M | F | M | F | F | F | F | M | F | M | M | F | M | |||||||||||
Fever | + | + | + | + | + | + | + | + | + | + | + | - | + | |||||||||||
Splenomegaly | + | + | + | + | + | + | + | + | + | + | + | + | + | |||||||||||
Cytopenia | + | + | + | + | + | + | + | + | + | + | + | + | + | |||||||||||
Hypertriglyceridemia | ND | + | + | + | + | + | + | ND | - | + | - | + | + | |||||||||||
Hypofibrinogenemia | + | - | + | + | + | + | - | + | - | + | + | + | + | |||||||||||
HSCT | Y | Y | N | Y | Y | N | N | Y | Y | Y | N | Y | Y | |||||||||||
Outcome | DoD | Alive | Alive | DoD | Alive | DoD | DoT | Alive | Alive | Alive | DoD | Alive | Alive |
UPN indicates unique patient number; HSCT, allogeneic hematopoietic stem cell transplantation; +, present; -, absent; ND, not determined; DoD, dead of disease; and DoT, dead of toxicity.