Table 3

Results of studies on the treatment of hemophagocytic lymphohistiocytosis in adult patients.

Authors	Characteristics	N	Cause	Treatment	Outcome	Etc
Imashuku et al. [60] (Japan)	Early vs. delayed etoposide	20	EBV-HLH	Etoposide (within 4 weeks)	Survivor: 5/7 vs. 1/13	2.5-yr OS:
Imashuku et al. [60] (Japan)	Early vs. delayed etoposide	20	EBV-HLH	Etoposide (within 4 weeks)	Survivor: 5/7 vs. 1/13	85 vs. 10%
Tseng et al. [63] (Taiwan)	Non-infectious vs. Infectious	96	Non-infection: 66	Observational study	Mortality: 70% vs. 47%
Tseng et al. [63] (Taiwan)	Non-infectious vs. Infectious	96	Infectious: 30	Observational study	Mortality: 70% vs. 47%
Buyse et al. [59] (France)	HLH at ICU	56	Tumor: 43	Etoposide: 45	Mortality: 29/56	MAHS
			Non-viral: 13	Corticosteroid: 31		Aggressive supportive care
			Viral: 10	IVIG: 3		Aggressive supportive care
Park et al. [61] (Korea)	HLH with hemophagocytosis	23	EBV: 16	HLH-94 or 2004: 13	Long-term survivor: 6/23 (26%)	4 survivors received alloHCT
			Idiopathic: 6	Immunosuppressive therapy: 9
			Hepatitis A: 1	Immunosuppressive therapy: 9
Yoon et al. [64] (Korea)	Non malignancy associated HLH	126	EEBV, infection, autoimmune	HLH-94 81 (64.3%)	CR: 64.3%	8-week treatment response is a predictor for survival
Shin et al. [62] (Korea)	CHOP-based Tx	17		CHOP	CR: 41.2%	2-year OS rate: 43.9%
Shin et al. [62] (Korea)	CHOP-based Tx	17		CHOP	PR: 17.6%	2-year OS rate: 43.9%

Abbreviations: CHOP, cyclophosphamide/doxorubicin/vincristine/prednisolone; CR, complete remission; EBV, EpsteinBarr virus; HLH, hemophagocytic lymphohistiocytosis; ICU, intensive care unit; IVIG, intravenous immunoglobulin; MAHS, malignancy-associated hemophagocytic syndrome; OS, overall survival; PR, partial remission.

Blood Res 2021;56:S17~S25 https://doi.org/10.5045/br.2021.2020323