Clinical Characteristics of the Patients With HLH
| Case No. . | Age/Sex . | Family History . | Etiology . | Fever . | Hepatomegaly . | Splenomegaly . | DIC . | Pancytopenia . | Liver Dysfunction . | Hyperferritinemia . | Hypertriglydemia . | BMT . | Outcome . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 1/F | − | Unknown | + | + | − | + | + | + | + | ND | Alive | |
| 2 | 3/F | − | Unknown | + | − | − | − | + | + | + | − | Alive | |
| 3 | 3/F | − | EBV | + | + | + | + | − | + | + | ND | Alive | |
| 4 | 13/F | − | Ki-lymphoma | + | − | − | + | + | + | + | ND | Alive | |
| 5 | 4/F | + | FHL | + | + | + | + | + | + | + | + | Allogeneic | Dead |
| 6 | 1/M | + | FHL | + | + | − | + | + | + | + | ND | Allogeneic | Dead |
| 7 | 0/M | + | FHL | + | + | + | + | + | + | + | + | Alive | |
| 8 | 1/F | + | FHL | + | + | + | + | + | + | + | + | Alive | |
| 9 | 1/M | + | FHL/EBV | + | + | + | + | + | + | + | + | Dead | |
| 10 | 1/F | + | FHL/EBV | + | + | + | + | + | + | + | + | Dead | |
| 11 | 2/F | − | EBV | + | + | + | + | + | + | + | + | Alive | |
| 12 | 0/M | − | EBV | + | + | + | + | + | + | + | + | Dead | |
| 13 | 1/F | − | Influenza AH3 | + | + | − | + | + | + | + | − | Alive | |
| 14 | 1/F | − | EBV | + | + | + | + | + | + | − | − | Alive | |
| 15 | 5/M | − | Unknown | + | + | + | + | + | + | + | + | Alive | |
| 16 | 10/F | − | Unknown | + | + | + | + | + | + | + | + | Alive | |
| 17 | 2/M | − | Unknown | + | + | + | − | + | + | + | − | Alive | |
| 18 | 7/F | − | EBV | + | + | − | + | + | + | + | − | Syngeneic | Alive |
| 19 | 4/F | − | EBV | + | + | − | + | + | + | + | − | Dead |
| Case No. . | Age/Sex . | Family History . | Etiology . | Fever . | Hepatomegaly . | Splenomegaly . | DIC . | Pancytopenia . | Liver Dysfunction . | Hyperferritinemia . | Hypertriglydemia . | BMT . | Outcome . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 1/F | − | Unknown | + | + | − | + | + | + | + | ND | Alive | |
| 2 | 3/F | − | Unknown | + | − | − | − | + | + | + | − | Alive | |
| 3 | 3/F | − | EBV | + | + | + | + | − | + | + | ND | Alive | |
| 4 | 13/F | − | Ki-lymphoma | + | − | − | + | + | + | + | ND | Alive | |
| 5 | 4/F | + | FHL | + | + | + | + | + | + | + | + | Allogeneic | Dead |
| 6 | 1/M | + | FHL | + | + | − | + | + | + | + | ND | Allogeneic | Dead |
| 7 | 0/M | + | FHL | + | + | + | + | + | + | + | + | Alive | |
| 8 | 1/F | + | FHL | + | + | + | + | + | + | + | + | Alive | |
| 9 | 1/M | + | FHL/EBV | + | + | + | + | + | + | + | + | Dead | |
| 10 | 1/F | + | FHL/EBV | + | + | + | + | + | + | + | + | Dead | |
| 11 | 2/F | − | EBV | + | + | + | + | + | + | + | + | Alive | |
| 12 | 0/M | − | EBV | + | + | + | + | + | + | + | + | Dead | |
| 13 | 1/F | − | Influenza AH3 | + | + | − | + | + | + | + | − | Alive | |
| 14 | 1/F | − | EBV | + | + | + | + | + | + | − | − | Alive | |
| 15 | 5/M | − | Unknown | + | + | + | + | + | + | + | + | Alive | |
| 16 | 10/F | − | Unknown | + | + | + | + | + | + | + | + | Alive | |
| 17 | 2/M | − | Unknown | + | + | + | − | + | + | + | − | Alive | |
| 18 | 7/F | − | EBV | + | + | − | + | + | + | + | − | Syngeneic | Alive |
| 19 | 4/F | − | EBV | + | + | − | + | + | + | + | − | Dead |
Abbreviations: BMT, bone marrow transplantation; ND, not determined.