Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Table 2 Characteristics of PLCH and therapies at diagnosis and during follow-up

Characteristic	At PLCH diagnosis n = 17	After the last ICU hospitalization n = 11^a	At last visit n = 11^a
Median interval after the PLCH diagnosis	_	63 days (range: 4–966)	11.2 years (range: 0.5–16)
Respiratory symptoms
No symptoms	3 (20)	2 (25)	9 (82)
NYHA 2	2 (13)	5 (38)	2 (18)
NYHA 3
NYHA 4	12 (71)	4 (36)
Oxygen requirement	12 (71)	4 (36)	0
LCH therapy
VLB Steroid	16 (94)	3 (27)
VP16 ARA-C		1 (9)
2CDA	1 (7)	5 (45)
Targeted therapy		2 (18)	2 (18)
DAS Score, Median [min, max]	5 [3,23]	3 [1, 3]	1 [1,1]
CT Scores,	(11 evaluated cases)	(7 evaluated cases)	(7 cases evaluated)
Cyst, median [min, max]	12 [0,24]	10 [0,21]	10 [0,18]
Nodule, median [min, max]	0 [0,6]	0 [0,3]	0 [0,2]

Values are the number of patients (%), unless indicated otherwise
—: not applicable
Abbreviations: DAS Disease Activity Score, CT computed tomography, ICU intensive care unit, LCH Langerhans cell histiocytosis, NYHA New York Heart Association, PLCH Pulmonary LCH, VLB vinblastine, VP16 Ara-C Etoposide-Cytarabine, 2CDA Cladribine
^a6 patients died in the last ICU

ISSN: 1750-1172