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KMID : 1035620180060030179
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Allergy Asthma & Respiratory Disease
2018 Volume.6 No. 3 p.179 ~ p.183
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Isolated pulmonary Langerhans cell histiocytosis in a 10-month-old infant
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Jang Hye-Jin
Kim Yoon-Hee
Kim Kyung-Won
Sohn Myung-Hyun
Lyu Chuhl-Joo
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Abstract
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Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation and accumulation of abnormal dendritic (Langerhans) cells in various organs. Pulmonary involvement, although rare in children, has been reported in 20%?50% of childhood cases of multisystem LCH. Isolated pulmonary LCH in children, especially in infants, is still rarer, but should be suspected in those with cystic lung disease. We report a case of a 10-month-old boy who presented with chronic dyspnea and whose chest computed tomography (CT) scan demonstrated cystic lesions. Lung biopsy established the diagnosis of LCH; microscopy revealed a background of lymphocytes and eosinophils with kidney-shaped abnormal cells. These abnormal cells were positive for S-100, CD207 (Langerin), and CD1a on immunohistochemical staining. Chemotherapy was administered using a cytotoxic agent (vinblastine) and a steroid. After 12 weeks of induction chemotherapy, although no significant change in cyst size was noted on chest CT, clinical symptoms improved. Consolidation chemotherapy was then administered for 1 year. Thereafter, chest CT findings demonstrated a significant decrease in cyst size and a significant increase in the volume of normal lung parenchyma. Therefore, aggressive treatment of isolated pulmonary LCH in infants with severe tissue destruction and symptoms seems warranted.
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KEYWORD
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Langerhans cell histiocytosis, Lung biopsy, Chemotherapy, Infant
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