TABLE 2.

Clinical 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
Cardiovascular involvement, n (%)	5	3	131	6	1	2	ns
	(55.6)	(50)	(47.8)	(54.5)	(33.3)	(25)
Central nervous system involvement, n (%)	3	0	183	5	1	2	<0.001
	(33.3)	0	(67) *	(45.5)	(33.3)	(25)
Bone involvement, n(%)	6	4	232	10	3	6	ns
	(66.7)	(66.7)	(85)	(90.9)	(100)	(75)
Cortical bone tracer uptake, n(%)	3	1	161	9	3	5	0.001
	(100)	(33.3) *	(94.7)	(90)	(100)	(100)
Lung involvement, n (%)	4	2	62	1	0	3	ns
	(44.4)	(33.3)	(22.7)	(9.1)	(0.0)	(37.5)
Kidney/retroperitoneum involvement, n (%)	6	0	155	10	1	4	0.009
	(66.7)	0	(56.7)	(90.9) *	(33.3)	(50)
Skin involvement, n (%)	3	5	77	3	0	4	0.004
	(33.3)	(83.3) *	(28.2)	(27.3)	(0.0)	(50)
Serosal involvement, n (%)	5	3	79	4	0	3	ns
	(55.6)	(50)	(28.9)	(36.4)	(0.0)	(37.5)
Reticuloendothelial system involvement, n (%)	1	1	14	3	0	2	0.01
	(11.1)	(16,6)	(5.1)	(27.3) *	(0.0)	(25)

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