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Early cardiac response is possible in stage IIIb cardiac AL amyloidosis and is associated with prolonged survival.
Blood ( IF 20.3 ) Pub Date : 2022-11-03 , DOI: 10.1182/blood.2022016348
Marco Basset 1, 2 , Paolo Milani 1, 2 , Andrea Foli 1, 2 , Mario Nuvolone 1, 2 , Pietro Benvenuti 1, 3 , Martina Nanci 1, 2 , Francesca Fabris 1, 2 , Claudia Bellofiore 1, 4 , Giampaolo Merlini 1, 2 , Giovanni Palladini 1, 2
Affiliation  

Patients with immunoglobulin light chain (AL) amyloidosis and stage IIIb cardiac involvement have a dismal outcome despite the introduction of novel treatments. However, a rapid hematologic response translates in better survival. We evaluated the impact of early cardiac response and its depth on outcome in 249 patients with newly diagnosed stage IIIb cardiac AL amyloidosis. Hematologic and cardiac responses were evaluated by intent to treat. After a median follow-up of 52 months, 219 (84%) patients died, and median survival was 4.2 months. The 30- and 90-day hematologic response rates were 22% (at least very good partial response [VGPR] in 9%) and 24% (at least VGPR in 15%), respectively. Early hematologic response resulted in better survival. At 90 days, 21 (8%) patients achieved a cardiac response (cardiac very good partial response [cardiac VGPR] in 12 cases and cardiac partial response [cardiac PR] in 9). At the 90-day landmark analysis, cardiac response resulted in longer survival (median, 54 months), also in those patients who have achieved at least VGPR (median, 62 vs 26 months, P = .011). Patients with cardiac VGPR had a longer survival than those with cardiac PR (median, 92 vs 24 months; P = .027), whereas patients without cardiac response had a poor survival (median, 6 months). A baseline difference of involved/uninvolved free light chains > 50 mg/L (odds ratio [OR], 0.21, P = .024) and a bone marrow plasma cell infiltrate > 10% (OR, 0.23, P = .040) were negative predictors of 90-day cardiac response. Early cardiac responses are rare but possible in stage IIIb AL amyloidosis and translate to longer survival.

中文翻译:

在 IIIb 期心脏 AL 淀粉样变性中,早期心脏反应是可能的,并且与延长的生存期相关。

尽管采用了新的治疗方法,但患有免疫球蛋白轻链 (AL) 淀粉样变性和 IIIb 期心脏受累的患者预后不佳。然而,快速的血液学反应意味着更好的生存。我们在 249 名新诊断的 IIIb 期心脏 AL 淀粉样变性患者中评估了早期心脏反应及其深度对结果的影响。通过意向治疗评估血液学和心脏反应。中位随访 52 个月后,219 名 (84%) 患者死亡,中位生存期为 4.2 个月。30 天和 90 天的血液学反应率分别为 22%(至少非常好的部分反应 [VGPR] 为 9%)和 24%(至少 VGPR 为 15%)。早期血液学反应导致更好的生存。在 90 天时,21 名 (8%) 患者达到心脏反应(12 例心脏部分反应非常好 [心脏 VGPR],9 例心脏部分反应 [心脏 PR])。在 90 天的里程碑分析中,心脏反应导致更长的生存期(中位数,54 个月),在那些至少达到 VGPR 的患者中也是如此(中位数,62 对 26 个月,P = .011)。心脏 VGPR 患者的生存期长于心脏 PR 患者(中位时间,92 vs 24 个月;P = .027),而无心脏反应的患者生存期较差(中位时间,6 个月)。受累/未受累游离轻链的基线差异 > 50 mg/L(比值比 [OR],0.21,P = .024)和骨髓浆细胞浸润 > 10%(OR,0.23,P = .040) 90 天心脏反应的阴性预测因子。
更新日期:2022-06-30
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