Clinical Characteristics of Pulmonary Histiocytosis X.

Hwang, Yong Il; Park, Gun Min; Yim, Jae Joon; Yoo, Chul Gyu; Lee, Choon Taek; Kim, Young Whan; Han, Sung Koo; Shim, Young Soo

doi:2001.51.4.346

Tuberc Respir Dis > Volume 51(4); 2001 > Article

Original Article

Tuberculosis and Respiratory Diseases 2001;51(4):346-353.
DOI: https://doi.org/10.4046/trd.2001.51.4.346 Published online October 1, 2001.

Clinical Characteristics of Pulmonary Histiocytosis X.

Yong Il Hwang, Gun Min Park, Jae Joon Yim, Chul Gyu Yoo, Choon Taek Lee, Young Whan Kim, Sung Koo Han, Young Soo Shim

Abstract

BACKGROUND
Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. METHOD: Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. RESULT: There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. CONCLUSION: In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

Key Words: Pulmonary histiocytosis X, Langerhans cell histiocytosis, Eosinophilic granuloma of lung, Clinical characteristics