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先前诊断为多发性骨髓瘤的患者中诊断出的轻链(AL)淀粉样变性的临床特征和治疗反应。

Clinical features and treatment response of light chain (AL) amyloidosis diagnosed in patients with previous diagnosis of multiple myeloma.

机构信息

Division of Hematology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.

出版信息

Mayo Clin Proc. 2010 Mar;85(3):232-8. doi: 10.4065/mcp.2009.0547.

Abstract

OBJECTIVE

To identify and assess the clinical features and treatment response of light chain (AL) amyloidosis diagnosed in patients with previous diagnosis of multiple myeloma (MM).

PATIENTS AND METHODS

From a prospectively maintained database, we identified 47 patients seen between January 1, 1990, and August 31, 2008, with a diagnosis of AL amyloidosis that was made at least 6 months after MM diagnosis; these patients form the study group.

RESULTS

Among the 47 patients, 36 developed typical features, 3 had atypical features, and 8 had an incidental finding of amyloidosis. Amyloid deposits were demonstrated in bone marrow, subcutaneous fat aspirate, or organ biopsy in 24, 19, and 12 patients, respectively. One organ was involved in 29 patients (62%), whereas 11 patients (23%) had involvement in more than one organ. At diagnosis of AL amyloidosis, treatment was changed or started in 22 patients, whereas the same treatment was continued in 21 patients, and no treatment data were available for the rest. The best hematologic response included partial response or better in 11 patients (23%) and stable disease in 18 patients (38%). Improvement in an organ was seen in 3 of the 21 evaluable patients. The median overall survival from diagnosis of AL amyloidosis was 9.1 months (95% confidence interval, 4-14). Of the 6 patients still alive, 2 underwent peripheral blood stem cell transplant, and none had cardiac involvement or involvement in more than one organ.

CONCLUSION

Delayed onset of AL amyloidosis is rarely seen in patients with MM and requires a high index of suspicion for prompt diagnosis. Outcome of these patients is poor, especially in the presence of cardiac involvement.

摘要

目的

确定和评估先前诊断为多发性骨髓瘤(MM)的患者中诊断为轻链(AL)淀粉样变性的临床特征和治疗反应。

患者和方法

从一个前瞻性维护的数据库中,我们确定了 47 名患者,他们在 1990 年 1 月 1 日至 2008 年 8 月 31 日之间接受了治疗,在 MM 诊断后至少 6 个月被诊断为 AL 淀粉样变性;这些患者构成了研究组。

结果

在 47 名患者中,36 名患者出现典型特征,3 名患者出现非典型特征,8 名患者偶然发现淀粉样变性。在 24 名、19 名和 12 名患者中分别在骨髓、皮下脂肪抽吸物或器官活检中显示出淀粉样沉积物。29 名患者(62%)有一个器官受累,11 名患者(23%)有一个以上器官受累。在诊断为 AL 淀粉样变性时,22 名患者改变或开始治疗,21 名患者继续使用相同的治疗方法,其余患者则无治疗数据。最佳血液学反应包括 11 名患者(23%)部分缓解或更好,18 名患者(38%)稳定疾病。在 21 名可评估患者中,有 3 名患者的器官得到改善。从诊断为 AL 淀粉样变性到中位总生存期为 9.1 个月(95%置信区间,4-14)。在仍存活的 6 名患者中,2 名患者接受了外周血干细胞移植,并且没有患者存在心脏受累或超过一个器官受累。

结论

MM 患者中很少见延迟发生的 AL 淀粉样变性,需要高度怀疑以便及时诊断。这些患者的预后较差,尤其是存在心脏受累的情况下。

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