Clinical and Biologic Characteristics of 14 Children With FHL at Diagnosis
| Characteristic . | Value . |
|---|---|
| Sex ratio (female/male) | 5/9 |
| Mean age at diagnosis, mo (range) | 16 (2-48) |
| Consanguinity (n) | 2 |
| Family history (n) | 5 |
| Initial clinical presentation (n) | |
| Hepatosplenomegaly | 14 |
| High fever | 14 |
| Skin rash | 9 |
| Neurologic symptoms | 8 |
| Initial biologic presentation (n) | |
| Pancytopenia | 14 |
| Low fibrinogen level | 14 |
| Hypertriglyceridemia | 14 |
| Hyponatremia | 14 |
| Hemophagocytosis on bone marrow aspirate (n) | 12 |
| Activated peripheral T lymphocytes (HLA-DR+/CD3+) | 11/11 |
| Characteristic . | Value . |
|---|---|
| Sex ratio (female/male) | 5/9 |
| Mean age at diagnosis, mo (range) | 16 (2-48) |
| Consanguinity (n) | 2 |
| Family history (n) | 5 |
| Initial clinical presentation (n) | |
| Hepatosplenomegaly | 14 |
| High fever | 14 |
| Skin rash | 9 |
| Neurologic symptoms | 8 |
| Initial biologic presentation (n) | |
| Pancytopenia | 14 |
| Low fibrinogen level | 14 |
| Hypertriglyceridemia | 14 |
| Hyponatremia | 14 |
| Hemophagocytosis on bone marrow aspirate (n) | 12 |
| Activated peripheral T lymphocytes (HLA-DR+/CD3+) | 11/11 |