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[原发性淀粉样变性病例]

[A case of primary amyloidosis].

作者信息

Ardashev V N, Potekhin N P, Rukavitsyn O A, Borisov A G, Malysheva S A

出版信息

Klin Med (Mosk). 2006;84(6):56-9.

PMID:16875072
Abstract

Diagnostics of primary (AL) amyloidosis is difficult enough; treatment of this disease in not less difficult or more adequate. Because of similarity of the pathogenesis of AL-amyloidosis and that of multiple myeloma, similar therapeutic regimens, directed towards depression of plasma cell dyscrasia, are used in both cases: administration of melphalan in various doses together with prednisolone, administration of vincristine, adriablastine and dexamethasone, as well as high-dose chemotherapy with melphalan and autologic stem cell transplantation. This therapeutic approach makes it possible to reach clinico-laboratory remission and prolong the life of patients with AL-amyloidosis. The article contains a case description of a patient with AL-amyloidosis, who underwent a successful high-dose melphalan therapy with subsequent autologic stem cell transplantation.

摘要

原发性(AL)淀粉样变性的诊断已经足够困难;而这种疾病的治疗难度也不低,效果也不尽人意。由于AL淀粉样变性与多发性骨髓瘤的发病机制相似,因此在这两种情况下都采用了类似的治疗方案,旨在抑制浆细胞异常增生:使用不同剂量的美法仑联合泼尼松龙,使用长春新碱、阿霉素和地塞米松,以及采用美法仑进行大剂量化疗和自体干细胞移植。这种治疗方法能够实现临床实验室缓解,并延长AL淀粉样变性患者的生命。本文包含了一名AL淀粉样变性患者的病例描述,该患者接受了成功的大剂量美法仑治疗及随后的自体干细胞移植。

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