Abstract
Prolonged survival of patients with previously fatal mailgnancies has led to the identification of late appearing toxic manifestations of therapeutic agents. This report presents two children with late onset pulmoanry fibrosis believed secondary to remote cyclophosphamide administration. A 4. 1/2 yr. old white female with acute lymphoblastic leukemia received 70 gm of cyclophosphamide over a 34 month period. Four years later she developed progressive pulmonary fibrosis with pleural thickening and effusions, bilateral apical pneumothorax, reduced AP diameter of chest and cor pulmonale. Over the next three years pulmonary function tests showed progressively decreasing function and she expired with hypoxia and hypercapnea. Autopsy revealed interstitial pulmonary fibrosis. A 3 1/2 year old male with Hodgkin's disease (lymphocytic predominence) received mantle x-ray therapy (2200R) followed by cyclophosphamide, 42 gm in fifty-one months. Six years later he developed cough, tachypnea, reduced exercise tolerance and weight loss. Chest x-ray revealed decreased AP diameter, interstitial fibrosis and pleural thickening. Pulmonary function tests revealed marked restrictive lung disease with severely diminished vital capacity. There was also EKG evidence of cor pulmonale. Microscopical examination of transbronchial lung biopsy showed interstitial fibrosis. Pulmonary function has decreased over subsequent two years and his vital capacity is now 24% of expected values.
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Newman, A., Alvarado, C. & Gross, S. PULMONARY FIBROSIS, A DELAYED COMPLICATION OF CYCLOPHOSPHAMIDE. Pediatr Res 11, 478 (1977). https://doi.org/10.1203/00006450-197704000-00648
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DOI: https://doi.org/10.1203/00006450-197704000-00648