Table 1.

The main clinical features of enrolled patients

	All (n = 63)	EBV-HLH (n = 22)	LAHS (n = 29)	FHL (n = 4)	Unclear (n = 8)
General
Gender (male/female), n	42/21	16/6	19/10	3/1	4/4
Median age (range), y	34 (18-78)	30.5 (18-57)	40 (18-78)	32 (18-60)	27.5 (20-36)
Clinical features
Fever, n	63	22	29	4	8
Neutropenia, n	59	19	28	4	8
Anemia, n	49	14	26	2	7
Thrombocytopenia, n	52	20	26	2	4
Splenomegaly, n	50	17	23	4	6
Hepatomegaly, n	25	7	14	2	2
Hypertriglyceridemia, n	27	9	16	1	1
Hypofibrinogen, n	33	8	17	2	6
Ferritin↑, n	63	22	29	4	8
Bilirubin↑, n	44	17	18	4	5
Alanine aminotransferase↑, n	48	18	22	6	2
Albumin↓, n	35	11	19	1	4
Soluble CD25↑, n	63	22	29	4	8
Previous treatment of HLH
Regimen	HLH-94 ± rituximab	HLH-94 ± rituximab	HLH-94	HLH-94	HLH-94
Weeks from initial therapy to DEP, median (range)	3 (2-28)	3 (2-23)	2 (2-5)	11.5 (3-28)	3.5 (2-24)

LAHS, lymphoma-associated HLH.