Hachem Mohamad Ali M, Nasreddine Ghadir M, Farhat Solay, Hammoud Zeinab M, Saad Firas, Saad Wajih A
Hematology and Oncology, Faculty of Medical Sciences, Lebanese University, Beirut, LBN.
Hematology and Oncology, Morristown Medical Center, Morristown, USA.
Cureus. 2024 Aug 1;16(8):e65960. doi: 10.7759/cureus.65960. eCollection 2024 Aug.
Systemic light chain amyloidosis is a rare and severe disorder characterized by amyloid fibril deposition in various tissues, often leading to organ failure. Early diagnosis is crucial but challenging due to diverse clinical manifestations. Our case report presents a complex case of systemic light chain amyloidosis in a 62-year-old patient with cardiac, renal, neurological, and gastrointestinal involvement. The patient's treatment with cyclophosphamide, bortezomib, dexamethasone, and intravenous daratumumab yielded significant improvement, aligning with recent studies. Following treatment, the patient improved from stage IV to stage II systemic light chain amyloidosis per the National Comprehensive Cancer Network (NCCN) guidelines, indicating a more favorable prognosis. Hence, the successful integration of daratumumab in our case underscores its potential as a valuable addition to the treatment regimen for advanced systemic light chain amyloidosis, showcasing significant improvements across multiple organ systems.
系统性轻链淀粉样变性是一种罕见且严重的疾病，其特征是淀粉样原纤维沉积于各种组织中，常导致器官衰竭。由于临床表现多样，早期诊断至关重要但具有挑战性。我们的病例报告展示了一名62岁系统性轻链淀粉样变性患者的复杂病例，该患者存在心脏、肾脏、神经和胃肠道受累情况。该患者接受环磷酰胺、硼替佐米、地塞米松和静脉注射达雷妥尤单抗治疗后取得了显著改善，这与近期研究结果相符。根据美国国立综合癌症网络（NCCN）指南，治疗后该患者从IV期系统性轻链淀粉样变性改善为II期，提示预后更佳。因此，在我们的病例中成功加入达雷妥尤单抗凸显了其作为晚期系统性轻链淀粉样变性治疗方案中一项有价值补充的潜力，展示了在多个器官系统中取得的显著改善。
