Cohen A S, Rubinow A, Anderson J J, Skinner M, Mason J H, Libbey C, Kayne H
Am J Med. 1987 Jun;82(6):1182-90. doi: 10.1016/0002-9343(87)90222-1.
Primary amyloidosis has a variable course, but is generally associated with a short life expectancy. To date, no specific therapy has been available. Fifty-three patients with AL amyloidosis seen between 1976 and 1983 were treated with colchicine, and their clinical course and survival were compared with that in 29 other patients seen between 1961 and 1973. Of the variables measured, the treatment, the patient's sex, and the time interval from diagnosis to referral of treatment were significantly associated with length of survival. Median survival for the colchicine-treated patients was 17 months, compared with six months for the non-colchicine-treated patients. A surprising finding was the longer life span in female patients (median eight months versus four and a half months in the non-colchicine-treated group, and 25.5 months versus 10 month in the colchicine-treated group). The study suggests that colchicine has improved the life expectancy in AL amyloidosis. Although it is not a specific therapy, it may be a reasonable form of adjunctive treatment in this complex disorder.
原发性淀粉样变性病程各异，但总体上与预期寿命较短相关。迄今为止，尚无特效疗法。对1976年至1983年间诊治的53例AL淀粉样变性患者采用秋水仙碱进行治疗，并将其临床病程及生存期与1961年至1973年间诊治的另外29例患者进行比较。在所测量的变量中，治疗方式、患者性别以及从诊断到开始治疗的时间间隔与生存期显著相关。接受秋水仙碱治疗的患者中位生存期为17个月，未接受秋水仙碱治疗的患者为6个月。一个惊人的发现是女性患者的生存期更长（未接受秋水仙碱治疗组中，女性中位生存期为8个月，男性为4.5个月；接受秋水仙碱治疗组中，女性为25.5个月，男性为10个月）。该研究表明，秋水仙碱可提高AL淀粉样变性患者的预期寿命。虽然它并非特效疗法，但在这种复杂疾病中可能是一种合理的辅助治疗方式。
