SIMPLE CLUES TO DIAGNOSE A RARE DISEASE OF PULMONARY LANGERHANS CELL HISTOCYTOSIS

Abstract

SESSION TITLE: Medical Student/Resident Tobacco Cessation and Prevention SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Langerhans cell histocytosis (LCH) commonly occurs in bone, dermis, lymph nodes, liver, spleen, and lungs. Pulmonary LCH (PLCH) is a rare disease occurring in 10% of LCH population and is seen in 3-5% of lung biopsies. (1) CASE PRESENTATION: A young female presented with 3 months history of persistent productive cough with minimal yellow to green color phlegm. She had symptoms of allergic sinusitis in past. No history of asthma. History of (Cystic fibrosis) CF gene carrier. Social history: current 5-pack year smoking. Family history was positive for lung cancer in grandmother, CF in sister. Physical Examination was normal.CTA Chest: innumerable thin walled cysts predominantly in upper lobes and mediastinal lymphadenopathy.Labs: VEG-F D: 58 (Normal), Alpha-1 antitrypsin: Normal.PFT: Normal.VATS guided open biopsy: revealed incidental respiratory bronchiolitis and cystic lesions with central fibrosis and mixed infiltrate of lymphoid aggregate, eosinophils, plasma cells and histocytes characteristic of late or fibrotic stage of Langerhans cell histocytosis. DISCUSSION: PLCH occurs in 20-40 years age group and most commonly associated with smoking. Patients with PCLH present with symptoms of cough, dyspnea and pleuritic pain with spontaneous pneumothorax. The cells express histocyte marker CD1 A-reactive Langerhans cells and contain Birbeck granules. The finding of interstitial fibrosis with cysts or nodules in upper lobes in a young smoker is characteristic of PLCH. (2) CONCLUSIONS: Patients with PLCH are more prone for pneumothorax and pulmonary hypertension. (3) Smoking cessation is main focus in management. Immunosuppressive therapies are of limited value. Reference #1: Canadian Respiratory Journal. 2010 May-Jun; 17(3): e55–e62 Reference #2: European Respiratory Journal. 2006 Jun;27(6):1272-85 Reference #3: American Journal of Respiratory Critical Care Med. 2000;161(1):216 DISCLOSURES: No relevant relationships by Shafia Beg, source=Web Response No relevant relationships by Shweta Kambali, source=Web Response