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Multiple myeloma-associated AL amyloidosis: is a distinctive therapeutic approach warranted?

机译:多发性骨髓瘤相关性AL淀粉样变性:是否需要独特的治疗方法?

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The natural history of multiple myeloma (MM) was revolutionized by the introduction of haematopoietic stem cell transplantation to the treatment armamentarium of this disease. Defined subgroups of MM patients (such as the elderly or dialysis-dependent) have required an individualized approach in order to minimize the transplant-related mortality. Little, however, is known about the management of 12-30% of MM patients with coexistent AL amyloidosis as the amyloidopathy is often overlooked and when recognized these patients commonly are excluded from clinical trials. While occult amyloidosis appears to have no impact on the toxicity and outcome of MM patients, the presence of symptomatic amyloidopathy clearly worsens their prognosis. Use of induction chemotherapy drugs that can cause further damage to the heart (Adriamycin), nervous system (Vincristine) or kidneys should be avoided as should lengthy delays in proceeding to autograft. Further, refining the transplant eligibility criteria for this subgroupof patients with co-existent amyloidopathy to include the number of organs involved and the degree of cardiac involvement (NYHA class, Troponins and NT-pro-BNP levels) along with melphalan dose-adjustment will minimize the treatment-related toxicity and mortality and possibly allow a reversal of the organ damage induced by the amyloidogenic light chain.
机译:通过将造血干细胞移植引入该疾病的治疗药库,彻底改变了多发性骨髓瘤(MM)的自然史。 MM患者的特定亚组(例如老年人或依赖透析的患者)需要个体化的方法,以最大程度地减少与移植相关的死亡率。然而,关于淀粉样变性病常常被忽视,并且当认识到这些患者通常被排除在临床试验之外,对于治疗12-30%并存AL淀粉样变性的MM患者的治疗知之甚少。虽然隐匿性淀粉样变性似乎对MM患者的毒性和预后没有影响,但症状性淀粉样变性的存在显然使他们的预后恶化。应避免使用可能对心脏(阿霉素),神经系统(长春新碱)或肾脏造成进一步损害的诱导化疗药物,否则应推迟进行自体移植的时间。此外,完善该共存淀粉样病患者亚组的移植资格标准,以包括累及的器官数量和心脏受累程度(NYHA类,肌钙蛋白和NT-pro-BNP水平)以及美法仑剂量调整将最小化与治疗有关的毒性和死亡率,并可能逆转由淀粉样蛋白形成的轻链引起的器官损伤。

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