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• W4387266003 abstract "SESSION TITLE: Lung Pathology Case Report Posters 10 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic multisystem disorder. ECD most commonly manifests as multifocal sclerotic lesions of the long bones demonstrating sheets of foamy histiocytes on biopsy, with or without histiocytic infiltration of extra-osseous tissues. It often also involves other organ systems such as the lungs, spleen, kidneys, skin and retroperitoneum. CASE PRESENTATION: 30-year-old female who presented with progressive dyspnea for over a year. She has a history of severe obstructive sleep apnea on therapeutic positive airway pressure therapy, allergic rhinitis, and Hashimoto's thyroiditis. She's a non-smoker, without history of pulmonary diseases. She complains of dyspnea at rest and worse with exertion. She also endorses a chronic cough, which was intermittently productive with yellow sputum and occasional hemoptysis. She had a significant decline in her exercise capacity over the last year as well. CT chest showed abnormal findings with diffuse smooth interlobular septal thickening with diffuse ground glass opacities. Imaging also noted splenomegaly. Furthermore, her echocardiogram showed a normal LV function. These findings persisted on a follow-up scan 3 months later with continued worsening symptoms. Due to the progression of her disease, a video assisted thoracic surgery was performed and the biopsy was diagnostic of Erdheim-Chester disease. The disease was advanced with pituitary, lung, and splenic involvement. After an extensive evaluation, molecular diagnostic evaluation and a bone marrow aspiration biopsy, her disease was BRAF mutation positive with no evidence bone marrow involvement. She was started on vemurafenib (BRAF kinase inhibitor) with rapid improvement of her symptoms. 2 weeks after initiation of therapy, she is reported no cough or shortness of breath and her oxygen requirements stabilized. A repeat CT scan 3 months after initiation of therapy showed improvement in pulmonary findings. DISCUSSION: Erheim-Chester disease (ECD) is a rare histiocytosis with diverse clinical manifestations, ranging from localized and indolent presentations to life-threatening multi-system disease. Recent advances have led to the approval of targeted treatment agent (vemurafenib) for BRAF-V600-mutant ECD. Although, previously considered an inflammatory, nonneoplastic disorder, ECD is now included in the WHO classification of hematopoietic tumors. Skeletal involvement occurs in up to 96% of ECD patients, however this was not seen in our case, making this an unusual finding because there was multi-system involvement without the most common manifestation of bone involvement. This can often present as bone pain and osteosclerosis of diametaphyseal regions of long bones. This contrasts with the osteolytic lesions seen in Langerhans cell histiocytosis. Pulmonary involvement is often seen on CT as interlobular septal thickening, diffuse, and localized centrilobular nodular opacities, ground glass opacities and fissural thickening. Similar findings were noted on CT in our case. Furthermore, ECD is generally seen in the 5th or 6th decade of life, which was unusual given that our patient was only 30 years old. CONCLUSIONS: ECD is a rare condition affecting multiple organ systems and requires a high degree of suspicion to diagnose and treat. Our case demonstrates the classic pulmonary findings of this disease without noting any bone involvement which is seen in most cases of this disease. REFERENCE #1: Gaurav Goyal, Mark L. Heaney, Matthew Collin, Fleur Cohen-Aubart, Augusto Vaglio, Benjamin H. Durham, Oshrat Hershkovitz-Rokah, Michael Girschikofsky, Eric D. Jacobsen, Kazuhiro Toyama, Aaron M. Goodman, Paul Hendrie, Xin-xin Cao, Juvianee I. Estrada-Veras, Ofer Shpilberg, André Abdo, Mineo Kurokawa, Lorenzo Dagna, Kenneth L. McClain, Roei D. Mazor, Jennifer Picarsic, Filip Janku, Ronald S. Go, Julien Haroche, Eli L. Diamond; Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era. Blood 2020; 135 (22): 1929–1945. doi: https://doi.org/10.1182/blood.2019003507 REFERENCE #2: Merai H, Collas D, Bhagat A, Mandalia U. Erdheim-Chester Disease: A Case Report and Review of the Literature. J Clin Imaging Sci. 2020 Jun 18;10:37. doi: 10.25259/JCIS_68_2020. PMID: 32637228; PMCID: PMC7332465. REFERENCE #3: Di Stefano, G., Granai, M., Giudici, F., Roselli, G., Lazzi, S., & Santi, R. (2021). Xanthomatous inflammatory infiltrate involving the spleen: An unusual presentation of Erdheim-Chester Disease and review of the literature. American Journal of Case Reports, 22. https://doi.org/10.12659/ajcr.931060 DISCLOSURES: No relevant relationships by Rao Afzal No relevant relationships by Nameer Al-Yousif No relevant relationships by Marvi Bukhari No relevant relationships by Zuhaab Khan No relevant relationships by Salahuddin Nasir No relevant relationships by Karen Selk" @default.
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• W4387266003 date "2023-10-01" @default.
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• W4387266003 title "A CHEST SPECIAL: A CASE OF ERDHEIM-CHESTER DISEASE" @default.
• W4387266003 doi "https://doi.org/10.1016/j.chest.2023.07.3129" @default.
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