Abstract：
AL amyloidosis is the most common form of systemic amyloidosis. However, the non-specific nature of presenting symptoms requires the need for a heightened clinical suspicion to detect unexplained manifestations in the appropriate clinical setting. Early detection and treatment are crucial as the degree of cardiac involvement emerges as a primary prognostic predictor of survival in a patient with AL amyloidosis. Following the diagnosis of AL amyloidosis with appropriate tissue biopsies, prompt treatment with a bortezomib, cyclophosphamide and dexamethasone-based first-line induction with or without daratumumab should be initiated. The goal of treatment is to achieve the best haematologic response possible, ideally with involved free light chain <20 mg/L, as it offers the best chance of organ function improvement. Treatment should be changed if patients do not achieve a partial response within 2 cycles of treatment or very good partial response after 4 cycles or after autologous stem cell transplant, as achievement of profound and prolonged clonal responses translates to better organ response and long-term outcomes. Early involvement of multidisciplinary subspecialists such as renal physicians, cardiologists, neurologists, and gastroenterologists for optimal maintenance and support of involved organs is recommended for optimal management of patients with AL amyloidosis.
摘要：
AL淀粉样变性是最常见的全身性淀粉样变性形式。然而,出现症状的非特异性性质要求需要加强临床怀疑,以在适当的临床环境中检测无法解释的表现.早期检测和治疗至关重要，因为心脏受累程度是AL淀粉样变性患者生存的主要预后预测指标。在用适当的组织活检诊断AL淀粉样变性后，用硼替佐米迅速治疗，在有或没有达雷妥单抗的情况下,应开始基于环磷酰胺和地塞米松的一线诱导.治疗的目标是实现可能的最佳血液学反应，理想情况下涉及游离轻链<20mg/L，因为它提供了器官功能改善的最佳机会。如果患者在2个治疗周期内没有达到部分反应，或者在4个周期或自体干细胞移植后没有达到非常好的部分反应，则应改变治疗方法。作为实现深刻和长期的克隆反应转化为更好的器官反应和长期结果。早期多学科专家如肾内科医师的参与，心脏病学家,神经学家,建议胃肠病学家对受累器官进行最佳维护和支持，以对AL淀粉样变性患者进行最佳管理。
