Thuong Vu Le, Minh Duc Nguyen, Tra My Thieu-Thi, Ba Tung Nguyen, Phuong Thuy Le Tu, Minh Thong Pham
Department of Internal Medicine, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Viet Nam.
Department of Radiology, Ha Noi Medical University, Ha Noi, Viet Nam.
Respir Med Case Rep. 2021 Mar 4;32:101377. doi: 10.1016/j.rmcr.2021.101377. eCollection 2021.
Primary laryngotracheobronchial amyloidosis is a rare pulmonary disease that can cause endobronchial stenosis. This disease has never previously been reported in Vietnam. We aimed to report a laryngotracheobronchial amyloidosis case in a 43-year-old female, which may be the first reported case in Vietnam. The patient had a 4-year history of progressive hoarseness, dyspnea, and hemoptysis. Multiple bronchial biopsies combined with detailed clinical information suggested an amyloidosis disease. Red congo staining was positive in bronchial samples, and a further workup found positive red congo staining in subcutaneous fatty tissue biopsy samples. Tracheostomy was performed due to severe dyspnea related to laryngeal stenosis. A multidisciplinary consultation was held, and chemotherapy with melphalan and dexamethasone were prescribed due to the systemic effects of the disease. After 2 cycles of chemotherapy, the patient showed improvement in dyspnea and cough. Due to the inexperience of both the clinicians and pathologists, this case was diagnosed quite late. In the future, if this diagnosis is considered in the differential diagnosis, an earlier diagnosis and better treatment outcome can be reached.
原发性喉气管支气管淀粉样变性是一种可导致支气管内狭窄的罕见肺部疾病。越南此前从未有过该病的报道。我们旨在报告一例43岁女性的喉气管支气管淀粉样变性病例,这可能是越南首例报道病例。该患者有4年渐进性声音嘶哑、呼吸困难和咯血病史。多次支气管活检结合详细的临床信息提示为淀粉样变性疾病。支气管样本刚果红染色呈阳性,进一步检查发现皮下脂肪组织活检样本刚果红染色也呈阳性。由于与喉狭窄相关的严重呼吸困难,患者接受了气管切开术。进行了多学科会诊,鉴于该疾病的全身影响,开出了美法仑和地塞米松化疗方案。经过2个周期的化疗,患者的呼吸困难和咳嗽症状有所改善。由于临床医生和病理学家经验不足,该病例诊断较晚。未来,如果在鉴别诊断中考虑到这一诊断,有望实现更早的诊断和更好的治疗效果。