Table 1.
Baseline characteristics and treatment outcomes of included patients (n = 88)
| Characteristic | Value |
|---|---|
| Gender (male), % | 50 (56.8%) |
| Age, years old | 1.4 (0.2–15) |
| Fever | 88 (100%) |
| CMV % | 13 (14.8%) |
| Leukocyte | 3.22 (0.25–36.47) |
| Neutrophil | 1.195 (0.05–11.86) |
| Platelet | 67.00 (7–562) |
| Hemoglobin | 86.5 (31–131) |
| Fibrinogen | 1.29 (0.27–4.56) |
| Albumin | 29.27 ± 5.61 |
| Ferritin | 3564.5 (132.0–591,123.0) |
| AST | 249.15 (21.0–9420.0) |
| ALT | 207.50 (8.0–4953.0) |
| LDH | 944.5 (156.0–5311.0) |
| TG | 3.34 (0.39–13.56) |
| CRP | 15.05 (0.25–161.54) |
| GGT | 207.5 (7.0–1550.0) |
| D-dimer | 2489.0 (129.0–35,600.0) |
| DIC % | 28 (31.8%) |
| Splenomegaly % | 49 (55.7%) |
| Jaundice % | 34 (38.6%) |
| Bone marrow involvement % | 51 (58.0%) |
| Hepatomegaly % | 65 (73.9%) |
| Ascites % | 21 (23.9%) |
|
Lymphadenopathy % CR achievement after treatments |
28 (31.8%) 58 (65.9%) |
| Therapy | |
| HLH94/2004based therapy | 34 (38.6%) |
| Steroid + IVIG | 8 (9.1%) |
| Steroid | 22 (25%) |
| Only observation | 23 (26.1%) |
| Hematopoietic stem cell transplantation | 1 (1.1%) |
| Causes of HLH | 56 (76.7%) |
| EBV-associated | 30 (33.7%) |
| Immunodeficiency | 6 (6.7%) |
| Autoimmune disease (AID) | 13 (14.6%) |
| Unknown cause and infection other than EBV | 40 (44.9%) |
| Treatment response at 8 weeks | |
| Early stable responder | 29 (33.3%) |
| Late stable responder | 28 (32.2%) |
| Unstable responder | 17 (19.5%) |
| Non-responder | 13 (14.9%) |
Abbreviations: HLH hemophagocytic lymphohistiocytosis, AST aspartate aminotransferase, ALT alanine transferase, LDH lactate dehydrogenase, CRP C-reactive protein, DIC disseminated intravascular coagulation, EBV Epstein-Barr virus, CR complete remission, EBV Epstein-Barr virus, TG triglyceride, GGT gamma-glutamyl transpeptidase