Gertz M A, Kyle R A
Dysproteinemia Clinic, Mayo Clinic, Rochester, MN 55905.
Arch Intern Med. 1990 Mar;150(3):629-33.
We followed up 153 patients with biopsy-proven primary systemic amyloidosis to determine their risk for acute nonlymphocytic leukemia or a dysmyelopoietic syndrome. In 10 patients cytogenetic abnormalities developed consistent with alkylator-induced damage to hematopoietic cells. In this group, the total melphalan dose ranged from 476 to 2450 mg (median, 1764 mg) administered over 21 to 92 months (median, 38 months). Eight of the 10 patients died as a direct result of pancytopenia, 1 died of progressive renal amyloid, and 1 remains alive with persistent complex cytogenetic abnormalities. Four patients had acute nonlymphocytic leukemia; 5 had a dysmyelopoietic syndrome; and 1 had a nondiagnostic bone marrow examination. Although only 6.5% of the entire group had leukemia or a dysmyelopoietic syndrome, the actuarial risk in patients surviving 3.5 years was 21%. Median survival from onset of dysmyelopoietic syndrome or acute leukemia was 8.1 months.
我们对153例经活检证实的原发性系统性淀粉样变性患者进行了随访，以确定他们患急性非淋巴细胞白血病或骨髓生成异常综合征的风险。10例患者出现了与烷化剂诱导的造血细胞损伤一致的细胞遗传学异常。在该组中，美法仑的总剂量在476至2450毫克之间（中位数为1764毫克），给药时间为21至92个月（中位数为38个月）。10例患者中有8例直接死于全血细胞减少症，1例死于进行性肾淀粉样变性，1例仍存活，伴有持续的复杂细胞遗传学异常。4例患者患有急性非淋巴细胞白血病；5例患有骨髓生成异常综合征；1例骨髓检查结果未明确诊断。虽然整个组中只有6.5%的患者患有白血病或骨髓生成异常综合征，但存活3.5年的患者的精算风险为21%。从骨髓生成异常综合征或急性白血病发病开始的中位生存期为8.1个月。
