Supportive Care and Symptom Management for Patients With Immunoglobulin Light Chain (AL) Amyloidosis.
Supportive Care and Symptom Management for Patients With Immunoglobulin Light Chain (AL) Amyloidosis.
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DOI：
10.3389/fonc.2022.907584
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发表时间：
2022
影响因子：
4.7
通讯作者：
Tuchman, Sascha A.
中科院分区：
文献类型：
--
作者：
Jensen, Christopher E.;Byku, Mirnela;Hladik, Gerald A.;Jain, Koyal;Traub, Rebecca E.;Tuchman, Sascha A.
免疫球蛋白轻链（AL）淀粉样变性是一种克隆性浆细胞疾病，其特征是淀粉样纤维在各种组织中沉积，导致终末器官损伤。肾脏或心脏受累是最常见的，尽管中枢神经系统以外的任何器官都可能发生淀粉样蛋白沉积，因此症状表现可能会有所不同。最初临床表现的变异性和微妙性可能导致诊断延迟，并且器官受累通常是相当晚期的，并且在诊断建立时有症状。此外，虽然器官功能可以通过浆细胞定向治疗改善，但这种改善落后于血液学反应。因此，高度有效的支持性治疗，包括症状管理，是必不可少的，以提高生活质量，并最大限度地提高治疗的耐受性和生存的可能性。考虑到疾病的系统性，临床医生之间的密切合作对于有效管理至关重要。
Immunoglobulin light chain (AL) amyloidosis is a disorder of clonal plasma cells characterized by deposition of amyloid fibrils in a variety of tissues, leading to end-organ injury. Renal or cardiac involvement is most common, though any organ outside the central nervous system can develop amyloid deposition, and symptomatic presentations may consequently vary. The variability and subtlety of initial clinical presentations may contribute to delayed diagnoses, and organ involvement is often quite advanced and symptomatic by the time a diagnosis is established. Additionally, while organ function can improve with plasma-cell-directed therapy, such improvement lags behind hematologic response. Consequently, highly effective supportive care, including symptom management, is essential to improve quality of life and to maximize both tolerance of therapy and likelihood of survival. Considering the systemic nature of the disease, close collaboration between clinicians is essential for effective management.
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