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Chronic respiratory failure slowly developed in two pediatric patients following long-term cyclophosphamide therapy for lymphocytic malignancy. One patient survived 12 years after acute lymphocytic leukemia was diagnosed but died of respiratory failure at age 16 still in initial remission; the other, a 12-year survivor of Hodgkin disease, has progressive deterioration of pulmonary function. Each patient received cyclophosphamide (less than 70 g) during the initial years of the disease. Autopsy of the first patient and lung biopsy in the second revealed severe pulmonary fibrosis. There was loss of compliance and a dramatic change in the shape of the thorax which produced a markedly reduced anteroposterior diameter in both patients, and recurrent pneumothoraces in one.

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