Smirnova A G, Smirnov A V, Zander A, Afanas'ev B V
Ter Arkh. 2010;82(10):61-4.
To evaluate the efficiency of chemotherapy with standard doses of melfalan and dexamethazone versus autologous peripheral hemopoietic cell transplantation (auto-PHCT) in patients with AL amyloidosis and to reveal poor prognostic factors.
Of 36 patients diagnosed as having AL-amylodosis, 17 patients underwent auto-PHCT, 11 patients received chemotherapy only; 8 patients died prior to treatment.
In patients with AL-amyloidosis after chemotherapy and autotransplantation, 3-year overall survival was 28 and 64%, respectively. Low somatic ECOG status and cardiac lesion were independent poor prognostic factors of the disease. The number of involved organs failed to affect overall survival.
Auto-PHCT may be proposed as first-line therapy for patients with AL-amyloidosis who have a somatic ECOG score of 0 to 2 and not more than 3 organs involved. Young patients who have a satisfactory somatic status and no benefit from autotransplantation may undergo auto-PHCT. It is expedient to use average-dose melfalan and dexamethasone when treating patients who are ineligible for high-dose chemotherapy.
评估美法仑和地塞米松标准剂量化疗与自体外周造血细胞移植（auto-PHCT）治疗AL淀粉样变性患者的疗效，并揭示不良预后因素。
36例诊断为AL淀粉样变性的患者中，17例行auto-PHCT，11例仅接受化疗；8例在治疗前死亡。
化疗和自体移植后的AL淀粉样变性患者，3年总生存率分别为28%和64%。体能状态差和心脏病变是该疾病独立的不良预后因素。受累器官数量未影响总生存率。
对于体能状态ECOG评分为0至2且受累器官不超过3个的AL淀粉样变性患者，可将auto-PHCT作为一线治疗方案。体能状态良好且自体移植无获益的年轻患者可进行auto-PHCT。对于不符合高剂量化疗条件的患者，使用平均剂量的美法仑和地塞米松进行治疗是适宜的。
