PEDIATRICS Vol. 89 No. 3 March 1992, pp. 466-469
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Lung Involvement in Langerhans' Cell Histiocytosis: Prevalence, Clinical Features, and Outcome

S. Y. Ha MRCP1, P. Helms MRCP2, M. Fletcher SRN2, V. Broadbent MRCP1, and J. Pritchard FRCP1

1 From the Departments of Haematology and Oncology, Hospital for Sick Children, London, England
2 From the Department of Respiratory Medicine, Hospital for Sick Children, London, England

In Langerhans' cell histiocytosis, the prognostic significance of pulmonary disease is controversial. The clinical and radiological features and lung function tests of Langerhans' cell histiocytosis patients presenting to a single tertiary referral center between 1981 and 1987 were reviewed. Age at diagnosis ranged from 2 weeks to 16 years (median 1.7 years) and the male-female ratio was 2.4:1. No child presented with lung involvement alone. In 18 (40%) of 45 patients with multisystem disease there was clinical and/or radiological evidence of lung pathology. Another 6 children (13%) with normal chest roentgenograms had abnormal lung function tests, suggesting subclinical ("occult") involvement. Those with overt lung disease tended to present at a younger than average age (median 0.6 years). The most common functional disturbance was reduced lung or respiratory compliance with reduced lung volumes. Patients with and without lung involvement showed a similar pattern of involvement of other organs, with skin and bone most commonly affected. Of the 45 children with multisystem disease, 38 (84%) survived 2 to 7 years after diagnosis; there was a similar proportion of deaths in children with and without lung involvement. It is concluded that lung involvement occurs in nearly half of young children with multisystem Langerhans' cell histiocytosis but does not adversely affect outcome.

Key Words: Langerhans' cell histiocytosis • lung disease

Submitted on August 8, 1990
Accepted on February 5, 1991


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