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首页> 外文期刊>Clinical advances in hematology & oncology: H&O >How we treat systemic light-chain amyloidosis.
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How we treat systemic light-chain amyloidosis.

机译：我们如何治疗系统性轻链淀粉样变性病。

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摘要

Systemic light-chain (AL) amyloidosis is a multisystem disease characterized by organ toxicity and damage due to monoclonal free light chains, which are produced by a neoplastic clone of plasma cells in bone marrow. Current treatment strategies target the clone in order to decrease the production of the pathologic light chains and thereby stop or reverse organ toxicity and damage. AL amyloidosis remains a formidable and often incurable disease despite treatment options that include corticosteroids, cytotoxic chemotherapy, risk-adapted melphalan, autologous hematopoietic stem cell transplantation, proteasome inhibitors, and immunomodulatory drugs. New and effective treatment approaches that can reverse the organ damage are urgently needed. Physicians and clinical staff should be aware of the importance of providing best supportive care to patients with advanced AL-related organ dysfunction, given the patients' often tenuous hemodynamics and fragile functional status. Organ transplantation has a role in selected clinical situations, and the treating hematologist should be aware of this sometimes-useful option.
机译：系统性轻链（AL）淀粉样变性病是一种多系统疾病，其特征是器官毒性和单克隆游离轻链造成的损害，这些游离轻链是骨髓中浆细胞的肿瘤性克隆产生的。当前的治疗策略以克隆为目标，以减少病理轻链的产生，从而停止或逆转器官毒性和损伤。尽管治疗选择包括皮​​质类固醇，细胞毒性化学疗法，适应风险的美法仑，自体造血干细胞移植，蛋白酶体抑制剂和免疫调节药物，但AL淀粉样变性病仍然是一种可怕且通常无法治愈的疾病。迫切需要能够逆转器官损伤的新的有效治疗方法。考虑到患者经常的血液动力学和脆弱的功能状态，医师和临床人员应意识到为晚期AL相关器官功能障碍患者提供最佳支持治疗的重要性。器官移植在特定的临床情况中起作用，并且治疗血液学家应意识到这种有时有用的选择。

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