TABLE 1.

General characteristics of Erdheim–Chester patients according to different genotypes.

	NRAS	KRAS	BRAF	MAP2K1	PIK3CA	Other	P value
	n = 9	n = 6	n = 273	n = 11	n = 3	n = 8
Male gender, n (%)	8 (88.9)	2 (33.3)	172 (65.6)	10 (90.9)	3 (100)	6 (75.0)	ns
Age, mean ± SD	59.9 ± 9.8	59.8 ± 9.4	52.2 ± 17.7	51.9 ± 11.6	31 ± 1.7	43.6 ± 28.1	ns
Pediatric patient, n (%)	0 (0.0)	0 (0.0)	16 (5.9)	0 (0.0)	0 (0.0)	2 (25.0)	ns
Multigene involvement, n (%)	0 (0.0)	3 (50) *	14 (5.1)	1 (9.1)	0 (0.0)	4 (50.0) *	0.001
Concomitant myeloid	3	4	26	1	0	2	0.001
or lymphoid neoplasms, n (%)	(33.3)	(66.7) *	(9.5)	(9.1)	0	(25)
Type of concomitant neoplasm, n (%)
Acute	1 (33.3)	0 (0.0)	6 (23.1)	0 (0.0)	0 (0.0)	1 (50.0)	ns
Chronic	3 (100.0)	4 (100.0)	22 (84.6)	1 (100.0)	0 (0.0)	1 (50.0)	ns
Lymphoid	0 (0.0)	0 (0.0)	3 (11.5)	0 (0.0)	0 (0.0)	0 (0.0)	ns
Myeloid	3 (100.0)	4 (100.0)	19 (73.1)	1 (0.0)	0 (0.0)	2 (100.0)	ns
Myelodysplasia	0 (0.0)	0 (0.0)	4 (15.4)	0 (0.0)	0 (0.0)	0 (0.0)	ns
Mixed Histiocytosis	1 (11.1)	0 (0.0)	24 (8.8)	1 (9.1)	0 (0.0)	0 (0.0)	ns
Shared mutation between ECD and neoplasm	3 (100.0) *	4 (100.0) *	1 (7.7)	0 (0.0)	0 (0.0)	2 (100.0) *	0.004

*Categories for which there is a statistically significant difference. Statistically significant p-values are in bold.