Table 1 Characteristics of the patients at the time of diagnosis of PLCH
From: Management and outcomes of pneumothorax in adult patients with Langerhans cell Histiocytosis
Characteristic | N = 43 |
|---|---|
Age, years, median, [IQR] | 26.5 [22.9–35.4] |
Male sex, n (%) | 26 (60%) |
Smoker, n (%) | 39 (91%) |
Ex-smoker | 4 (9%) |
Cannabisa, n (%) | 14 (33%) |
Asymptomatic | 12 (28%) |
Respiratory symptoms, n (%) | 25 (58%) |
Cough | 17 (40%) |
Dyspnoea | 14 (33%) |
NYHA II/III | 13/1 |
Constitutional symptoms | 11 (26%) |
Histological diagnosis, n (%) | 33 (77%) |
Genotyping, n | 22 |
BRAFV600E, n (%) | 11 (50%) |
Pneumothorax as initial manifestation | 28 (65%) |
Isolated PLCH, n (%) | 30 (70%) |
MS LCH, n (%) | 13 (30%) |
Non-pulmonary LCH localizations, n | |
Bone | 4 |
Pituitary stalk | 9 |
Diabetes insipidus | 9 |
Anterior hypophysis dysfunction | 4 |
Skin | 4 |
Peripheral lymph nodes | 2 |
CNS | 1 |
Liver | 1 |
Lung functionb, n | 36 |
TLC, % of predicted, n = 33 | 94 [87–107] |
VC, % of predicted, n = 35 | 80 [65–96] |
RV/TLC, % of predicted, n = 29 | 141 [118–162] |
FEV1, % of predicted, n = 36 | 75.5 [59–90.5] |
FEV1/FVC %, n = 31 | 78 [70–84] |
DLCO % of predicted, n = 21 | 58 [53–71] |
Restrictionc, (%) | 3 (9%) |
Obstruction, (%) | 7 (23%) |
Air trapping, n (%) | 20 (69%) |