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. 2022 Sep 6;10:941318. doi: 10.3389/fped.2022.941318

Table 2.

Treatment and treatment outcomes in HLH patients with different subtypes.

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.