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Diffuse Lung Disease |

Pulmonary Langerhans Cell Histiocytosis Presenting as Recurrent Spontaneous Pneumothorax in an Adolescent Male

Swapnil Mehta*, MD; Dhruva Chaudhry, DM; Sanjay Fotedar, MD; Viral Sangwan, MD
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PGIMS, Rohtak, Rohtak, India


Chest. 2012;142(4_MeetingAbstracts):465A. doi:10.1378/chest.1390175
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Abstract

SESSION TYPE: ILD Global Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary langerhans cell histiocytosis is a rare cause of ILD that occurs predominantly in adults and is often associated with cigarette smoking. PLCH is a clinical variant of langerhans cell histiocytosis(LCH), disease is characterized by proliferation and infiltration of various body organs by langerhans cells. Organ systems involved in LCH include skin, bone, pituitary gland, lymph nodes and lungs.(1) Besides the parenchymal lesion involvement of pleura presents as Pneumothorax which may be recurrent adding the morbidity and mortality.(2) The disease is characterized by typical imaging characteristics in proper clinical context however, confirmatory diagnosis require lung biopsy and immunohistochemical staining for CD1a, HLA-DR and S-100 protein.3

CASE PRESENTATION: A 19-year-old male presented with sudden onset increasing in severity of breathlessness and left sided chest pain. He had previous history of non-productive cough, progressive dyspnea, with 3 episodes of SPN for which he was managed accordingly and put on WHO cat.-I antitubercular treatment. Suspecting pulmonary tuberculosis as underlying cause. Patient also gave history of 3 pack yr. Cigarette smoking. Physical examination showed diminished breath sounds on the left side. A chest radiograph showed multiple pulmonary nodules and a left side pneumothorax requiring urgent tube thoracostomy. Patient responded to tube thoracostomy and subsequently a pulmonary function testing was performed, which revealed obstructive lung disease. In view of recurrent pneumothorax, a high resolution CT scan of thorax was performed which showed normal to increased lung volume with multiple thin walled cystic lesions bilaterally with predominance in upper and middle lobes and relative sparing of lower lobes. In view of typical radiological findings, patient subsequently underwent open lung biopsy and staining for CD1-a, CD-68 and S-100 which came out positive yielding a histopathological diagnosis of PLCH.

DISCUSSION: PLCH is a rare disease involving dendritic cells and is associated with significant morbidity and mortality. In majority of patients cough, dyspnea, chest pain, weight loss and fever are the common presenting symptoms. Though Pneumothorax occurs in 25% of the patients, presentation with pneumothorax is uncommon and recurrent pneumothorax is still rare. Various genetic, occupational risk factors have been suggested which result in uncontrolled immune response involving Langerhan's cells. Cigarette smoking induces generation of bombesin like peptide from neuro-endocrine cells in pulmonary epithelium and in turn lung fibrosis. Cigarette smoking may directly activate langerhan's cells to secrete TNF-α and GM-CSF and antigen present in cigarette smoker may directly promote Langerhan's cells and T lymphocytes and local inflammation. Radiological features consist of small ill defined nodules varying from 1mm to 10 mm reticular or nodular opacities particularly involving upper or mid zone with basal sparing. As the disease progress cyst predominant and nodules tend to occur less frequently. HRCT is sensitive and useful in diagnostic evaluation of PLCH the predominant findings include nodules and thin walled cyst and is virtually diagnostic of PLCH. Basal sparing is a useful distinguishing feature from lymphangioleiomyomatosis and other cystic lung diseases the presentation varies with the stage of the disease.

CONCLUSIONS: PLCH should be considered in patients with normal radiographs after resolution of Pneumothorax.Various factors play important role in the disease course and management which include smoking cessation,drugs etc. Various chemotherapeutic agents have been used in PLCH involvement with variable success. Pneumothorax known to occur in PLCH and its recurrence are better managed with tube thoracostomy and pleurodesis then chest tube drainage alone. Patients with progressive pulmonary disease are the potential candidates for lung transplant although it is known to recur after lung transplant.

1) Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans-cell histiocytosis. N Engl J Med 2000;342:1969-1978

2) Lacronique J, Roth C, Battesti JP, Basset F, Chretien J. Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. Thorax. 1982 Feb; 37(2):104-109

3) Banchereau J, Briere F, Caux C, et al. Immunobiology of dendritic cells. Annu Rev Immunol 2000; 18: 767-811.

DISCLOSURE: The following authors have nothing to disclose: Swapnil Mehta, Dhruva Chaudhry, Sanjay Fotedar, Viral Sangwan

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PGIMS, Rohtak, Rohtak, India

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