Clinical features and treatment outcomes of pediatric Langerhans cell histiocytosis with macrophage activation syndrome-hemophagocytic lymphohistiocytosis

Table 1 Comparison of clinical-biological characteristics in MS-LCH patients with (n = 28) or without (n = 209) MAS-HLH in this study

Clinical characteristics	MS-LCH	Without MAS-HLH	With MAS-HLH	P values
n	237	209	28
Sex
Male	141 (59.5%)	126 (60.3%)	15 (53.6%)	0.542
Female	96 (40.5%)	83 (39.7%)	13 (46.4%)
Age at diagnosis (years)
≥ 2	105 (44.3%)	105 (50.2%)	0	< 0.001
< 2	132 (55.7%)	104 (49.8%)	28 (100%)
Median (range)	1.70 (0.10–14.95)	2.0 (0.10–14.95)	1.0 (0.20–1.78)	< 0.001
Bone involvement
No	40 (16.9%)	34 (16.3%)	6 (21.4%)	0.590
Yes	197 (83.1%)	175 (83.7%)	22 (78.6%)
Skin involvement
No	106 (44.7%)	102 (48.8%)	4 (14.3%)	< 0.001
Yes	131 (55.3%)	107 (51.2%)	24 (85.7%)
Liver involvement
No	152 (64.1%)	147 (70.3%)	5 (17.9%)	< 0.001
Yes	85 (35.9%)	62 (29.7%)	23 (82.1%)
Spleen involvement
No	183 (77.2%)	180 (86.1%)	3 (10.7%)	< 0.001
Yes	54 (22.8%)	29 (13.9%)	25 (89.3%)
Hematologic involvement
No	199 (84.0%)	199 (95.2%)	0	< 0.001
Yes	38 (16.0%)	10 (4.8%)	28 (100%)
Lung involvement
No	158 (66.7%)	141 (67.5%)	17 (60.7%)	0.524
Yes	79 (33.3%)	68 (32.5%)	11 (39.3%)
Pituitary involvement
No	186 (78.5%)	163 (78.0%)	23 (82.1%)	0.807
Yes	51 (21.5%)	46 (22.0%)	5 (17.9%)
Lymph nodes involvement
No	188 (79.3%)	172 (82.3%)	16 (57.1%)	0.005
Yes	49 (20.7%)	37 (17.7%)	12 (42.9%)
BRAF-V600E in lesion tissues^a
Total available samples	160	135	25
Negative	52 (32.5%)	49 (36.3%)	3 (12.0%)	0.019
Positive	108 (67.5%)	86 (63.7%)	22 (88.0%)
BRAF-V600E in plasma cell-free DNA^a
Total available samples	164	143	21
Negative	75 (45.7%)	73 (51.0%)	2 (9.5%)	< 0.001
Positive	89 (54.3%)	70 (49.0%)	19 (90.5%)

MS-LCH, multisystem Langerhans cell histiocytosis; MAS, macrophage activation syndrome; HLH, hemophagocytic lymphohistiocytosis
^aBRAF-V600E mutation in biopsies of lesion tissue or plasma cell-free DNA was determined in 160 and 164 MS-LCH patients respectively

ISSN: 1750-1172