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• W4387248272 abstract "SESSION TITLE: Diffuse Lung Disease: Truly Unique Cases SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/11/2023 09:40 am - 10:25 am INTRODUCTION: Lymphangioleiomyomatosis (LAM) is a rare progressive multisystem disease predominately affecting women of reproductive age, characterized by the spread and uncontrolled proliferation of smooth muscle-like cells in various organs-primarily lungs, kidneys and lymphatics. The most common presenting symptom is dyspnea. We present an unusual case of post-coital hemoptysis as the initial clinical feature of LAM subsequently diagnosed by transbronchial cryobiopsy. CASE PRESENTATION: A 47-year-old female with past history of laryngopharyngeal reflux, insulin-dependent diabetes mellitus presented to the pulmonary office for evaluation of sporadic hemoptysis of two years duration, post-coital in nature with increasing frequency. She was a lifetime smoker. She had been remotely seen for post-viral cough syndrome after COVID-19 which resolved spontaneously and denied any dyspnea or respiratory complaints otherwise, including history of pneumothorax or effusion. HRCT chest was performed which demonstrated findings of cystic lung disease versus emphysematous changes and few scattered nodules measuring up to 5 mm. Alpha-1-antitrypsin testing was normal. Pulmonary function testing was performed demonstrating normal spirometry and mildly reduced DLCO.She underwent bronchoscopy with transbronchial lung cryobiopsy. Histopathology revealed cyst formation associated with recent and old hemorrhage, with HMB-45 and desmin staining. Diagnosis was consistent with LAM.CT abdomen and pelvis was ordered and pending. VEGF-D resulted elevated at 960. The patient was referred to an expert LAM center in the interim. DISCUSSION: Lymphangioleiomyomatosis is a rare multisystem disease most often affecting young women, grouped amongst the family of neoplasms involving perivascular epitheliod differentiation. Sporadic LAM is terminology reserved for patients who do not exhibit Tuberous Sclerosis Complex (TSC). Abnormal proliferation of LAM cells is driven by mutations in TSC genes, notably TSC2. In sporadic LAM, factors like estrogen and growth factors may contribute to cellular proliferation, including differentiation through melanoma gene antigen expression, migration mediated by estrogen, immune system evasion, lymphangiogenesis via vascular endothelial growth factors C and D, and tissue destruction. Regardless of pathophysiology, the result is that of defective or deficient tuberin or hamartin in afflicted cells and subsequent dysregulated cell signaling through the mTOR pathway. As such, treatment for progressive disease involves use of sirolimus, an mTOR inhibitor with cytostatic properties. For mild disease, supportive care is often sufficient. The American Thoracic Society and Japanese Respiratory Society detail clinical practice guidelines that recommend transbronchial lung biopsy before surgical lung biopsy in situations where a diagnosis is required in patients with parenchymal lung cysts on HRCT but no other additional confirmatory features [2]. In our case, transbronchial lung cryobiopsy was employed in an effort to mitigate crush artifact or small specimen size, yielding positive tissue diagnosis. CONCLUSIONS: The incidence of LAM is rare. While most presentations are nonspecific and include fatigue or dyspnea, more overt findings include spontaneous pneumothorax or effusion. Interestingly, hemoptysis occurs infrequently, and post-coital hemoptysis only documented in a singular case report [1, 3]. Even in the absence of neurological/neuropsychiatric abnormalities, the diagnosis of LAM should remain in the differential in a patient presenting with post-coital hemoptysis and suggestive radiographic findings. Transbronchial cryobiopsy appears a useful tool for obtaining a histopathological diagnosis. REFERENCE #1: Badawi RA, Geddes DM. Exertional haemoptysis: LAM and TSC. Thorax. 2003;58(5):460. doi:10.1136/thorax.58.5.460 REFERENCE #2: Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2017;196(10):1337-1348. doi:10.1164/rccm.201709-1965ST REFERENCE #3: Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006;173(1):105-111. doi:10.1164/rccm.200409-1298OC DISCLOSURES: No relevant relationships by PABLO BEJARANO No relevant relationships by Nydia Martinez No relevant relationships by Ivan Romero-Legro No relevant relationships by Veronica Williams" @default.
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• W4387248272 date "2023-10-01" @default.
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• W4387248272 title "MARY HAD A LITTLE LAM: A CASE OF POST-COITAL HEMOPTYSIS" @default.
• W4387248272 doi "https://doi.org/10.1016/j.chest.2023.07.2080" @default.
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