Table 2.
Primary HLH (n = 9) | IAHS (n = 38) | MAS (n = 21) | M-HLH (n = 8) | p-value | |
---|---|---|---|---|---|
Treatment (n, %) | |||||
Pulse IVMP monotherapy | 0 (0) | 0 (0) | 3 (14) | 0 (0) | 0.042* |
IVIG monotherapy | 0 (0) | 12 (32) | 0 (0) | 0 (0) | 0.005* |
Combined therapy without etoposide | 1 (11) | 10 (26) | 17 (81) | 0 (0) | <0.001* |
Etoposide-combined therapy | 8 (89) | 16 (42) | 1 (5) | 4 (50) | <0.001* |
Chemotherapya | 0 (0) | 0 (0) | 0 (0) | 4 (50) | <0.001* |
Treatment outcome ( n , %) | |||||
Mortality | 5 (56) | 12 (32) | 5 (24) | 7 (88) | 0.007* |
Early mortality | 3 (33) | 5 (13) | 4 (19) | 1 (13) | 0.502 |
Early treatment response | 6 (67) | 24 (63) | 17 (81) | 2 (25) | 0.05 |
HLH, hemophagocytic lymphohistiocytosis; IAHS, infection-associated hemophagocytic lymphohistiocytosis; IVIG, intravenous immunoglobulin; IVMP, intravenous methylprednisolone, MAS, macrophage activation syndrome; M-HLH, malignancy-associated HLH. *p <0.05 was considered statistically significant. aChemotherapy according to underlying malignancies.