Majolino I, Marcenò R, Pecoraro G, Scimé R, Vasta S, Liberti G, Rizzo A, Indovina A, Caronia F
Dipartimento di Ematologia, Ospedale V. Cervello, Palermo, Italy.
Haematologica. 1993 Jan-Feb;78(1):68-71.
A 53-yr.-old woman with amyloidosis AL was treated with high-dose chemotherapy and autologous stem cell infusion in an attempt to suppress the amyloid secretion. A diagnosis of MGUS had been made six years earlier. During the last year her disease had progressively shifted to a full-blown picture of amyloidosis AL, with renal failure, proteinuria, renal amyloid deposition and plasma cell sheets in the marrow. After an unsuccessful attempt with standard-dose chemotherapy, she received a high-dose regimen of busulphan (14 mg/Kg) and melphalan (40 mg/m2), followed by the infusion of both autologous bone marrow and peripheral blood stem cells. She had full and prompt engraftment, but eight weeks post-graft developed interstitial pneumonitis: CMV was isolated. The patient died while in the intensive care unit. In the literature, this is the first case of amyloidosis AL treated with high-dose therapy and autologous transplantation.
一名53岁的原发性轻链型淀粉样变性病女性患者接受了大剂量化疗及自体干细胞输注,以抑制淀粉样蛋白分泌。6年前曾诊断为意义未明的单克隆丙种球蛋白病。在过去一年中,其病情逐渐发展为典型的原发性轻链型淀粉样变性病,出现肾衰竭、蛋白尿、肾脏淀粉样沉积以及骨髓浆细胞片。在标准剂量化疗失败后,她接受了白消安(14 mg/Kg)和美法仑(40 mg/m²)的大剂量方案治疗,随后输注了自体骨髓和外周血干细胞。她实现了快速且完全的植入,但移植后8周发生了间质性肺炎,分离出巨细胞病毒。患者在重症监护病房死亡。在文献中,这是首例接受大剂量治疗及自体移植的原发性轻链型淀粉样变性病病例。