Vial G, Lafargue A, Mercié P, Duffau P, Ribeiro E
Department of Internal Medicine and Clinical Immunology, Saint Andre Hospital, University Hospital Centre of Bordeaux, 1, rue Jean Burguet, 33000 Bordeaux, France.
Department of Clinical Gerontology, Saint Andre Hospital, University Hospital Centre of Bordeaux, 1, rue Jean Burguet, 33000 Bordeaux, France.
Rev Med Interne. 2022 Nov;43(11):673-676. doi: 10.1016/j.revmed.2022.07.013. Epub 2022 Aug 5.
Systemic immunoglobulin light-chain (AL) amyloidosis is characterized by deposition of amyloid fibrils of light chains produced by clonal CD38+plasma cells, resulting in organ dysfunction. Cardiac involvement has a major prognostic value. Antiplasma cell chemotherapy reduces the synthesis of immunoglobulin light chains (precursors of amyloid deposits). We describe a case of AL amyloidosis in a 95-year-old patient. Our patient responded poorly to treatment with rituximab, cyclophosphamide-bortezomib-dexamethasone, and rituximab-bendamustine. Finally, the anti-CD38 antibody daratumumab was associated with the best hematologic responsiveness without significant adverse effects. In conclusion, our case suggests that daratumumab is an effective and well-tolerated alternative to chemotherapy in the treatment af AL amyloidosis in very elderly patients.
系统性免疫球蛋白轻链（AL）淀粉样变性的特征是由克隆性CD38 +浆细胞产生的轻链淀粉样纤维沉积，导致器官功能障碍。心脏受累具有重要的预后价值。抗浆细胞化疗可减少免疫球蛋白轻链（淀粉样沉积物的前体）的合成。我们描述了一例95岁的AL淀粉样变性患者。我们的患者对利妥昔单抗、环磷酰胺-硼替佐米-地塞米松以及利妥昔单抗-苯达莫司汀治疗反应不佳。最后，抗CD38抗体达雷妥尤单抗产生了最佳的血液学反应，且无明显不良反应。总之，我们的病例表明，在治疗高龄患者的AL淀粉样变性时，达雷妥尤单抗是一种有效且耐受性良好的化疗替代方案。
