Table 3 Summary of clinical data of 37... | American Society of Hematology

Table 3

Summary of clinical data of 37 patients with FHL5

	Value
No. of patients	37
Ethnic origin
White, n	14*
Turkey, n	14
Middle East, n	7
Far East Asian, n	2
Male:female	17:20
Median age at diagnosis (range)	3 mo (3 d to 19 y)
≥ 5 HLH criteria fulfilled†	32/37
Abnormal NK degranulation	13/16
CNS involvement	15/27
Treatment with HLH 94/2004	26/34
HSCT	26/36
Median age at HSCT (range)	1 y (4 m to 24 y)
Outcome alive	22/36
Alive without HSCT	2‡
Death from HLH	8
Alive after HSCT	20
Death from TRM	6

	Value
No. of patients	37
Ethnic origin
White, n	14*
Turkey, n	14
Middle East, n	7
Far East Asian, n	2
Male:female	17:20
Median age at diagnosis (range)	3 mo (3 d to 19 y)
≥ 5 HLH criteria fulfilled†	32/37
Abnormal NK degranulation	13/16
CNS involvement	15/27
Treatment with HLH 94/2004	26/34
HSCT	26/36
Median age at HSCT (range)	1 y (4 m to 24 y)
Outcome alive	22/36
Alive without HSCT	2‡
Death from HLH	8
Alive after HSCT	20
Death from TRM	6

TRM indicates transplant-related mortality.

In 1 of these patients, the father was white and the mother was Far East Asian.

In the 5 patients who did not fulfill ≥ 5 criteria, reporting on criteria was incomplete.

Both patients are currently listed for HSCT.