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53 例多发性骨髓瘤合并轻链淀粉样变性患者心脏受累的临床分析。

Clinical Analysis of Cardiac Involvement in 53 Patients With Multiple Myeloma Coexistent With Light Chain Amyloidosis.

机构信息

Multiple Myeloma Medical Center of Beijing, Department of Hematology, Beijing Chao-yang Hospital, Capital Medical University, Beijing, China.

Multiple Myeloma Medical Center of Beijing, Department of Hematology, Beijing Chao-yang Hospital, Capital Medical University, Beijing, China.

出版信息

Clin Lymphoma Myeloma Leuk. 2020 Aug;20(8):519-525.e1. doi: 10.1016/j.clml.2020.01.002. Epub 2020 Mar 19.

Abstract

BACKGROUND

We identified 53 patients with multiple myeloma (MM) who had biopsy evidence of light chain amyloidosis (AL), and studied their cardiac involvement and outcomes.

PATIENTS AND METHODS

Our cohort consisted of 53 patients in whom MM and AL were initially diagnosed from July 1, 2006 to June 30, 2016.The diagnosis of MM required > 10% of clonal plasma cells in bone marrow and 1 of the CRAB symptoms, meanwhile, the diagnosis of AL must meet pathologic diagnostic criteria and monoclonal immunoglobulin light chain. Echocardiograms and cardiac biomarker such as N terminal pro B-type natriuretic peptide was used for evaluation of cardiac damage on the baseline and before every cycle of the regimen.

RESULTS

There were 36 men and 17 women with a median age of 59 years; their main organ involvement was kidney (72%) and heart (62%). Of these, 22 patients were treated with a bortezomib-based regimen, and the response rate was more effective than the other 21 patients who received non-bortezomib-based regimens (64% vs. 29%). The median overall survival (OS) for the total cohort was 12 months (P < .05). The median OS of the MM cohort with International Staging System stage I and II together was 34 months, which was longer than that of patients with stage III of 8 months. The median OS in Mayo stages I, II, and III was 38, 8, and 1 months, respectively (P < .05). Cardiac involvement significantly adversely affected survival (6 vs. 40 months), as did systolic blood pressure (< 90 mmHg, 3 vs. 8.5 months).

CONCLUSIONS

Patients coexistent with MM and AL is rare; AL has a negative impact on survival for the total cohort. Especially, cardiovascular dysfunction caused by AL maybe a major determinant of shortening survival.

摘要

背景

我们鉴定了 53 例多发性骨髓瘤(MM)患者,这些患者的组织活检证实存在轻链淀粉样变性(AL),并对其心脏受累情况和结局进行了研究。

患者和方法

我们的队列包括 53 例于 2006 年 7 月 1 日至 2016 年 6 月 30 日期间初次诊断为 MM 和 AL 的患者。MM 的诊断需要骨髓中克隆性浆细胞 >10%,以及 CRAB 症状之一,同时,AL 的诊断必须符合病理诊断标准和单克隆免疫球蛋白轻链。基线和每个治疗周期前均使用超声心动图和心脏生物标志物(如 N 末端 pro B 型利钠肽)评估心脏损伤情况。

结果

患者中男 36 例,女 17 例,中位年龄 59 岁;主要器官受累为肾脏(72%)和心脏(62%)。其中,22 例接受硼替佐米为基础的方案治疗,缓解率高于接受非硼替佐米为基础方案治疗的 21 例患者(64%比 29%)。总队列的中位总生存期(OS)为 12 个月(P<0.05)。国际分期系统(ISS)I 期和 II 期 MM 患者的中位 OS 为 34 个月,长于 III 期患者的 8 个月。Mayo 分期 I、II 和 III 期患者的中位 OS 分别为 38、8 和 1 个月(P<0.05)。心脏受累显著影响生存(6 个月比 40 个月),收缩压<90mmHg(<8.5 个月)也是如此。

结论

同时患有 MM 和 AL 的患者罕见;AL 对总队列的生存有负面影响。特别是,AL 引起的心血管功能障碍可能是缩短生存的主要决定因素。

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