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%) |