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Obstructive Lung Diseases |

Case of Rapidly Progressive Pulmonary Langerhans Cell Histiocytosis in a Nonsmoker

Kimber Foust, MD; Gregory Fishbein, MD; Scott Genshaft, MD; Tisha Wang, MD
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UCLA, Los Angeles, CA


Chest. 2013;144(4_MeetingAbstracts):666A. doi:10.1378/chest.1704447
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Abstract

SESSION TITLE: Interstitial Lung Disease Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare lung disease that is generally, but not invariably, associated with cigarette smoking. [1] Typically young adults present with dry cough and shortness of breath and the prognosis can be favorable if it is diagnosed early. [1] We report an unusual case of a young female non-smoker with rapidly progressive PLCH leading to respiratory failure and death prior to a definitive diagnosis.

CASE PRESENTATION: A 22-year-old previously healthy female was referred to pulmonary clinic for evaluation of shortness of breath and cough that had been slowly progressive over several months. She was otherwise fully functional and attended her college graduation only a week prior to presentation. She had no history of smoking, connective tissue disease, or noxious exposures and physical exam was only revealing for a few inspiratory rhonchi. Initial labs including a blood counts and metabolic panels, HIV test, and rheumatologic serologies were unremarkable. Pulmonary function testing revealed a very severe restrictive ventilatory defect with moderate diffusion impairment and a walk test revealed desaturations to 85%. Chest imaging was obtained showing extensive bilateral cystic lung lesions (fig. 1), and a surgical lung biopsy was recommended for a definitive diagnosis. Within 10 days of her initial evaluation, she required admission for worsening hypoxemia. Empiric pulse steroids and an urgent lung transplant evaluation were initiated. Two days later, she required intubation for respiratory failure followed by a subsequent cardiac arrest. Despite bilateral chest tubes, vasopressors, and an attempt at extracorporeal membrane oxygenation (ECMO), she expired. A limited autopsy of her lungs revealed cystic changes on gross examination. Microscopically, there was dense cellular inflammation within and surrounding bronchi and bronchioles. Immunohistochemical stains showed the inflammatory infiltrate to be strongly positive for CD1a and S100 consistent with Langerhans cells. (Fig. 2)

DISCUSSION: PLCH is a rare interstitial lung disease with the characteristic findings of the accumulation of Langerhans cells in the small airways leading to the formation of nodules, initially cellular, but often progressing to completely fibrotic nodules. [1] Pulmonary involvement in LCH may occur alone or as part of a multisystem disorder. [1] The gold standard for definitive diagnosis is surgical lung biopsy, [2] and smoking cessation is essential in the treatment of PLCH [1]

CONCLUSIONS: Overall prognosis for PLCH appears to be good though a subgroup exists that develops chronically progressive disease.

Reference #1: Vassallo, R., et al., Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. The New England journal of medicine, 2002. 346(7): p. 484-90.

Reference #2: Vassallo, R., Diffuse lung diseases in cigarette smokers. Seminars in respiratory and critical care medicine, 2012. 33(5): p. 533-42.

DISCLOSURE: The following authors have nothing to disclose: Kimber Foust, Gregory Fishbein, Scott Genshaft, Tisha Wang

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