Lévy Y, Belghiti-Deprez D, Sobel A
Immuno-pathologie clinique, Hôpital Henri Mondor, Créteil.
Ann Med Interne (Paris). 1988;139(3):190-3.
AL amyloidosis is a serious complication of monoclonal gammopathy. The therapeutic strategy in amyloidosis associated with myeloma is to decrease the amyloidogenic precursor synthetised by the monoclonal plasmocytic proliferation. However, when systemic amyloidosis complicates a so called "benign" monoclonal gammopathy, this therapeutic approach is debatable. We report 10 cases of AL amyloidosis without myeloma treated by chemotherapy. Eight patients were initially given alkylating agents (cyclophosphamide or melphalan) which had no effect on the clinical progression of their systemic amyloidosis or on the plasma concentrations of the precursor. A limited open clinical trial including 4 patients was then undertaken based on the Vincristine, Adriamycine, Dexamethasone combination recently proposed for cases of resistant myeloma. A 50% reduction in the serum monoclonal protein was observed in 2 patients with this treatment. However, the mean survival of the 10 patients (25 months) was not longer than that previously reported for patients receiving more conventional treatment. The results of this limited trial indicate the need for further controlled therapeutic trials with larger numbers of patients in order to assess the effect of polychemotherapy in patients with AL amyloidosis.
AL淀粉样变性是单克隆丙种球蛋白病的一种严重并发症。与骨髓瘤相关的淀粉样变性的治疗策略是减少由单克隆浆细胞增殖合成的淀粉样蛋白前体。然而，当系统性淀粉样变性并发所谓的“良性”单克隆丙种球蛋白病时，这种治疗方法存在争议。我们报告了10例接受化疗的非骨髓瘤性AL淀粉样变性病例。8例患者最初接受了烷化剂（环磷酰胺或美法仑）治疗，这对其系统性淀粉样变性的临床进展或前体的血浆浓度均无影响。随后，基于最近为耐药骨髓瘤病例提出的长春新碱、阿霉素、地塞米松联合方案，对4例患者进行了一项有限的开放临床试验。2例接受该治疗的患者血清单克隆蛋白降低了50%。然而，这10例患者的平均生存期（25个月）并不长于先前报道的接受更传统治疗的患者。这项有限试验的结果表明，需要进行更多患者参与的进一步对照治疗试验，以评估联合化疗对AL淀粉样变性患者的疗效。
