Yella Prashanth Reddy, Jagani Prachi P, Jagani Ravi P, Skaria Priya Elsa, Chandra Abhinav
Internal Medicine/Hospital Medicine, Yuma Regional Medical Center, Yuma, USA.
Pre-Medical Sciences, Richmond Gabriel University, Kingstown, VCT.
Cureus. 2024 Aug 16;16(8):e67013. doi: 10.7759/cureus.67013. eCollection 2024 Aug.
Primary or light-chain (AL) (lambda) amyloidosis is a rare systemic disorder that is characterized by the misfolding of autologous proteins and the extracellular deposition of abnormally folded proteins composed of immunoglobulin light chains, often caused by plasma cell dyscrasias. We present a unique case of a 57-year-old female with multiple comorbidities, including extensive smoking history and chronic kidney disease, who was incidentally discovered to have a left upper lobe lung nodule on a chest X-ray prompted by complaints of shortness of breath. The patient underwent biopsy of the lung nodule, and by utilizing the gold standard diagnostic technique of a Congo red stain, positive test results confirmed the diagnosis of AL amyloidosis. However, additional investigations, including bone marrow and fat pad biopsies, were negative for plasma cell dyscrasias. The patient subsequently underwent a wedge resection of the nodule, and a follow-up positron emission tomography-computed tomography (PET-CT) scan showed only post-surgical changes in the left upper lobe of the lung without evidence of disease progression or systemic involvement. Given the asymptomatic and multisystem symptomology of most cases, treatment options for AL amyloidosis are individualized. This case discusses pulmonary nodular AL amyloidosis and highlights the diagnostic and treatment options for this disorder.
原发性或轻链(AL)(λ)淀粉样变性是一种罕见的系统性疾病,其特征是自体蛋白质错误折叠以及由免疫球蛋白轻链组成的异常折叠蛋白质在细胞外沉积,通常由浆细胞发育异常引起。我们报告了一例独特的病例,一名57岁女性,有多种合并症,包括广泛的吸烟史和慢性肾脏病,因呼吸急促就诊,胸部X线检查偶然发现左上肺叶有一个肺结节。患者接受了肺结节活检,通过使用刚果红染色这一金标准诊断技术,阳性检测结果确诊为AL淀粉样变性。然而,包括骨髓和脂肪垫活检在内的其他检查未发现浆细胞发育异常。患者随后接受了结节楔形切除术,后续的正电子发射断层扫描-计算机断层扫描(PET-CT)显示仅左肺上叶有术后改变,无疾病进展或全身受累迹象。鉴于大多数病例无症状且有多系统症状,AL淀粉样变性的治疗方案是个体化的。本病例讨论了肺结节性AL淀粉样变性,并强调了该疾病的诊断和治疗选择。