Table 1. Clinical features of two groups of adult PLCH.
| Case | Isolated pulmonary group | Extrapulmonary recidivism group | |||||
|---|---|---|---|---|---|---|---|
| 1 | 2 | 3 | 4 | 5 | 6 | ||
| Gender | M | M | M | F | M | M | |
| Age (years) | 20 | 24 | 35 | 53 | 42 | 64 | |
| Clinical manifestations | Cough and difficulty breathing, bilateral pneumothorax | Cough, sputum | Cough, sputum, difficulty breathing | Recurrent spontaneous pneumothorax | Chest pain | Chest pain | |
| Smoking (number per day, years) | 20, 7 | 3, 0.5 | NA | 0 | 20, 20 | 40, 40 | |
| CT scan | Thin-walled cysts, bilateral pneumothorax | Thin-walled cysts, micro-nodules (<0.3 cm) | Big-nodules, partial cystic changes | Thin-walled cysts, nodules (0.1–0.8 cm) | Nodules (0.3–1 cm) | Nodules (<1 cm), central cystic change | |
| Other organ | No | No | No | Skin, thyroid | 4th rib, pituitary | 3rd rib | |
| Underlying disease | No | No | Aml | Ptc, Scid | No | No | |
| Treatment | Sc, Op 3 month | Sc | Cvp 3 cycles | Thymalfasin, 2 months (t1w) | Sc | Etoposide 15 cycles (q1w), Ecop 3 cycles (q4w) | |
| Follow-up (months) | 35 | 7 | 8 | 38 | 46 | 19 | |
| Prognosis | Alive | Alive | Dead | Alive | Dead | Alive | |
PLCH, pulmonary Langerhans cell histiocytosis; F, female; M, male; NA, not available; AML, acute mononuclear leukemia; PTC, papillary thyroid carcinoma; SCID, severe combination of immunodeficiency; OP, oral prednisone; SC, smoking cessation; CVP, cyclophosphamide, vincristine and prednisone; t1w, two times per weeks; q1w, one time per week; q4w, one time per four weeks; ECOP, etoposide, cyclophosphamide, vincristine, predniso.