Allergy Asthma Respir Dis.  2018 May;6(3):179-183. 10.4168/aard.2018.6.3.179.

Isolated pulmonary Langerhans cell histiocytosis in a 10-month-old infant

• Jang H
• Kim YH
• Kim KW
• Sohn MH
• Lyu CJ

• KMID: 2412522
• DOI: http://doi.org/10.4168/aard.2018.6.3.179

Abstract

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.

Keyword

Langerhans cell histiocytosis; Lung biopsy; Chemotherapy; Infant

MeSH Terms

Biopsy
Child
Consolidation Chemotherapy
Diagnosis
Drug Therapy
Dyspnea
Eosinophils
Histiocytosis, Langerhans-Cell*
Humans
Induction Chemotherapy
Infant*
Lung
Lung Diseases
Lymphocytes
Male
Microscopy
Thorax
Tomography, X-Ray Computed

Figure

• 

  Fig. 1. A chest x-ray revealed that lung base has increased lucency due to cystic change, hyperinflated lungs with a reticular pattern.

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  Fig. 2. (A) A chest computed tomography (CT) at the level of the lung bases shows multiple thin-walled cysts with hardly any normal intervening lung parenchyma in the lower lobes. (B) A chest CT at the level of the upper lobes shows multiple thin-walled cyst. (C, D) A chest CT was performed after induction chemotherapy including vinblastine and steroid for response assessment. (C) A chest CT present increased size and number of multiple cystic lesions in Rt. lower lobe and decreased size in Lt. lower lobe. (D) A chest CT present increased size of cyst in both upper lung. (E, F) A chest CT was performed after consolidation chemotherapy for response assessment. The range and size of the cyst significantly decreased, and the volume of the normal lung parenchyma increased.

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  Fig. 3. (A) Peribronchial cellular infiltrate composed of neoplastic cells with histiocytoid features (H&E, ×100). (B) On high-power magnification the neoplastic cells are seen to display kidney shaped groove nuclear and cytoplasmic features of Langerhans cell (H&E, ×200). (C) A panel of microphotographs; CD1a cytoplasmic and membranous positivity on immunohistochemical stain (CD1a, ×200). (D) S100 positivity in the atypical histiocytes on LBC cytospin smears (S100, ×200).

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