The prognostic value of diagnosing concurrent multiple myeloma in immunoglobulin light chain amyloidosis
The prognostic value of diagnosing concurrent multiple myeloma in immunoglobulin light chain amyloidosis
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DOI：
10.1111/bjh.12269
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发表时间：
2013-05-01
影响因子：
6.5
通讯作者：
Liedtke, Michaela
中科院分区：
文献类型：
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作者：
Dinner, Shira;Witteles, Wesley;Liedtke, Michaela
在免疫球蛋白轻链淀粉样变性（AL）患者中，根据现行国际骨髓瘤工作组（IMWG）标准定义的症状性或无症状性多发性骨髓瘤（MM）合并诊断的患病率和预后价值尚不清楚。我们研究了46例连续AL患者进行定量血清M-蛋白和克隆骨髓浆细胞，以及MM的终末器官损伤的综合评价。使用标准形态学和CD 138免疫组化染色，57%和80%的患者被发现有并发MM，分别。9例患者表现出与症状性MM诊断一致的终末器官损害。虽然伴或不伴并发骨髓瘤的AL患者的总生存率相似（1年总生存率68% vs. 87%; P=0，中心点27），但症状性骨髓瘤的诊断与较差的结局相关（1年总生存率39% vs. 81%; P=0，中心点005）。通过标准形态学和CD 138免疫组化定量骨髓浆细胞，发现AL患者中并发MM的患病率远高于既往报告。评估骨髓浆细胞浸润和骨髓瘤相关终末器官损害的存在可能对AL患者的诊断有临床意义。
The prevalence and prognostic value of a concomitant diagnosis of symptomatic or asymptomatic multiple myeloma (MM), as defined by the current International Myeloma Working Group (IMWG) criteria, in patients with immunoglobulin light chain amyloidosis (AL), are unknown. We studied 46 consecutive patients with AL who underwent quantification of serum M-protein and clonal bone marrow plasma cells, as well as a comprehensive evaluation for end organ damage by MM. Using standard morphology and CD138 immunohistochemical staining, 57% and 80% of patients were found to have concomitant MM, respectively. Nine patients exhibited end organ damage consistent with a diagnosis of symptomatic MM. While overall survival was similar between AL patients with or without concurrent myeloma (1-year overall survival 68% vs. 87%; P=0 center dot 27), a diagnosis of symptomatic myeloma was associated with inferior outcome (1-year overall survival 39% vs. 81%; P=0 center dot 005). Quantification of bone marrow plasma cells by both standard morphology and CD138 immunohistochemistry identified a much higher prevalence of concurrent MM in patients with AL than previously reported. Evaluation of bone marrow plasma cell infiltration and presence of myeloma associated end organ damage could be clinically useful for prognostication of patients with AL.