[Long-term follow up study of four cases of pulmonary Langerhans' cell histiocytosis].
We reported the clinical features of 4 cases with pulmonary Langerhans' cell histiocytosis. All of them were men who had a history of smoking. They ranged in age from 23 to 46 years. Cases 1 and 3 did not stop smoking, while Cases 2 and 4 did stop smoking. All of the chest CTs revealed small nodules and cysts, and during the follow-up period of 8 to 13 years, the numbers of nodules decreased and the walls of the cysts became thin in all of the cases. Their pulmonary function tests revealed restrictive impairment in 3 cases and reduced diffusion capacity in 2 cases, but none showed obstructive impairment. FEV1% and DL(CO) deteriorated during the follow-up period. Case 1 died of respiratory failure after 8 years. Cases 2 and 4 were both alive with home oxygen therapy after 11 and 12 years, respectively. Case 3 was alive but complained of dyspnea on exertion after 13 years. In conclusion, all of the 4 cases showed deterioration of pulmonary function, and one of them died due to respiratory failure. The prognosis of patients with pulmonary Langerhans' cell histiocytosis appears to be poor.
Nishio
Chihiro
C
Department of Respiratory Medicine, National Hospital Organization Minami-Kyoto Hospital.
Nakahara
Yasuharu
Y
Mochizuki
Yoshiro
Y
Kawamura
Tetsuharu
T
Sasaki
Shin
S
Tsukamoto
Hiroaki
H
Mayumi
Tetsuichiro
T
Kobashi
Yoichiro
Y
D000328:Adult; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012907:Smoking / Q000009:adverse effects