SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare tobacco-associated interstitial lung disease causing cysts (1). PLCH is defined by the peribronchiolar accumulation of Langerhan cells, a dendritic cell found in respiratory epithelium (2). Constitutively activated Langerhans cells lead to increased and dysregulated inflammation resulting in the characteristic appearance of (peribronchiolar) nodules progressing to cyst formation (2). Pathologic diagnosis is often confirmed by the presence of S100 protein, CD1a positive, or CD207 (langerin) (3). When Langerhan Cell Histiocytosis is confined to the lungs, 90% of cases are related to tobacco exposure (2). Continued exposure to tobacco will often result in worsening disease. We present an unusual case of spontaneous remission of PLCH while persistent exposure to cannabis and tobacco. CASE PRESENTATION: A forty-four-year-old female current tobacco smoker 30 pack years, cannabis user presented to our hospital with nausea, dyspnea, and weight loss. Her medical history was significant for heavy tobacco and cannabis use. Initial exam revealed bilateral rhonchi though she was breathing comfortably on room air. Computer tomography (CT) of the chest revealed bilateral pulmonary nodules, some with cavitiation, and involvement predominantly in the upper lobes (Figure 1). Transthoracic biopsy (Figure 1) showed eosinophils, fibrosis, and tobacco-pigmented macrophages. Immunohistochemically stains focally positive for S-100 and HLA-DR-CD1a, confirming the diagnosis of PLCH. The patient was counseled on cessation of tobacco and cannabis inhalation, but did not follow up as an outpatient. On subsequent admission for gastrointestinal issues, CT scan demonstrated resolution of most of the nodules with residual small cysts despite persistent use of tobacco and cannabis. DISCUSSION: This case of spontaneous remission without cessation of tobacco or cannabis, represents an unusual trajectory in the disease course of PLCH. Treatments of PLCH centers around avoidance of toxin inhalation (tobacco and cannabis) and supportive care for pulmonary complications (1). The cytotoxic medication Cladribine (purine antagonist) has been used effectively in severe cases. Recently recognized, PLCH has been associated with up to 50% CD1a positive cells with mitogen kinase BRAFV600E mutations, which may lend itself to future targeted therapy. The spontaneous remission presented may support limited therapeutic intervention for PLCH, though randomized control trials are lacking. ten months after her initial diagnosis the pulmonary department continues to follow and support this patient and emphasize tobacco cessation. CONCLUSIONS: Pulmonary Langerhan Cell Histiocytosis is a rare interstitial lung disease linked to tobacco that follows a variable disease trajectory and requires an individualized approach in the absence of high quality clinical trials. Reference #1: Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX. Diffuse Cystic Lung Disease. Part I. American journal of respiratory and critical care medicine. 2015;191(12):1354-66. Reference #2: Richards JC, Lynch DA, Chung JH. Cystic and nodular lung disease. Clinics in chest medicine. 2015;36(2):299-312, ix. Reference #3: Roden AC, Yi ES. Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective. Archives of pathology & laboratory medicine. 2016;140(3):230-40. DISCLOSURES: No relevant relationships by Elliott Bondi, source=Web Response No relevant relationships by Joshua Davidson, source=Web Response No relevant relationships by Volha Liaukovich, source=Web Response No relevant relationships by Edith Okoye, source=Web Response No relevant relationships by Edith Okoye, source=Web Response No relevant relationships by Erum Zahid, source=Web Response
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