The main clinical features of enrolled patients
| . | All (n = 63) . | EBV-HLH (n = 22) . | LAHS (n = 29) . | FHL (n = 4) . | Unclear (n = 8) . |
|---|---|---|---|---|---|
| General | |||||
| Gender (male/female), n | 42/21 | 16/6 | 19/10 | 3/1 | 4/4 |
| Median age (range), y | 34 (18-78) | 30.5 (18-57) | 40 (18-78) | 32 (18-60) | 27.5 (20-36) |
| Clinical features | |||||
| Fever, n | 63 | 22 | 29 | 4 | 8 |
| Neutropenia, n | 59 | 19 | 28 | 4 | 8 |
| Anemia, n | 49 | 14 | 26 | 2 | 7 |
| Thrombocytopenia, n | 52 | 20 | 26 | 2 | 4 |
| Splenomegaly, n | 50 | 17 | 23 | 4 | 6 |
| Hepatomegaly, n | 25 | 7 | 14 | 2 | 2 |
| Hypertriglyceridemia, n | 27 | 9 | 16 | 1 | 1 |
| Hypofibrinogen, n | 33 | 8 | 17 | 2 | 6 |
| Ferritin↑, n | 63 | 22 | 29 | 4 | 8 |
| Bilirubin↑, n | 44 | 17 | 18 | 4 | 5 |
| Alanine aminotransferase↑, n | 48 | 18 | 22 | 6 | 2 |
| Albumin↓, n | 35 | 11 | 19 | 1 | 4 |
| Soluble CD25↑, n | 63 | 22 | 29 | 4 | 8 |
| Previous treatment of HLH | |||||
| Regimen | HLH-94 ± rituximab | HLH-94 ± rituximab | HLH-94 | HLH-94 | HLH-94 |
| Weeks from initial therapy to DEP, median (range) | 3 (2-28) | 3 (2-23) | 2 (2-5) | 11.5 (3-28) | 3.5 (2-24) |
| . | All (n = 63) . | EBV-HLH (n = 22) . | LAHS (n = 29) . | FHL (n = 4) . | Unclear (n = 8) . |
|---|---|---|---|---|---|
| General | |||||
| Gender (male/female), n | 42/21 | 16/6 | 19/10 | 3/1 | 4/4 |
| Median age (range), y | 34 (18-78) | 30.5 (18-57) | 40 (18-78) | 32 (18-60) | 27.5 (20-36) |
| Clinical features | |||||
| Fever, n | 63 | 22 | 29 | 4 | 8 |
| Neutropenia, n | 59 | 19 | 28 | 4 | 8 |
| Anemia, n | 49 | 14 | 26 | 2 | 7 |
| Thrombocytopenia, n | 52 | 20 | 26 | 2 | 4 |
| Splenomegaly, n | 50 | 17 | 23 | 4 | 6 |
| Hepatomegaly, n | 25 | 7 | 14 | 2 | 2 |
| Hypertriglyceridemia, n | 27 | 9 | 16 | 1 | 1 |
| Hypofibrinogen, n | 33 | 8 | 17 | 2 | 6 |
| Ferritin↑, n | 63 | 22 | 29 | 4 | 8 |
| Bilirubin↑, n | 44 | 17 | 18 | 4 | 5 |
| Alanine aminotransferase↑, n | 48 | 18 | 22 | 6 | 2 |
| Albumin↓, n | 35 | 11 | 19 | 1 | 4 |
| Soluble CD25↑, n | 63 | 22 | 29 | 4 | 8 |
| Previous treatment of HLH | |||||
| Regimen | HLH-94 ± rituximab | HLH-94 ± rituximab | HLH-94 | HLH-94 | HLH-94 |
| Weeks from initial therapy to DEP, median (range) | 3 (2-28) | 3 (2-23) | 2 (2-5) | 11.5 (3-28) | 3.5 (2-24) |
LAHS, lymphoma-associated HLH.