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与慢性淋巴细胞白血病/小淋巴细胞淋巴瘤相关的系统性淀粉样变性。

Systemic amyloidosis associated with chronic lymphocytic leukemia/small lymphocytic lymphoma.

机构信息

Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.

出版信息

Am J Hematol. 2013 May;88(5):375-8. doi: 10.1002/ajh.23413. Epub 2013 Mar 19.

Abstract

To clarify the presentation and course of patients with chronic lymphocytic leukemia (CLL) and amyloidosis. Mayo databases were interrogated for patients who carried a diagnosis of amyloidosis and CLL evaluated at Mayo Clinic, Rochester from January 1974 to October 2012. Charts were abstracted and data analyzed. Of the 33 patients identified, 20 (61%) were diagnosed with AL and 13 (39%) with non-AL. Only four patients had immunoglobulin light chain amyloidosis (AL) that could be solely attributed to the CLL clone; another six had both a plasma cell clone and a CLL clone that shared the same light chain. Median overall survival was 15.6 months for patients with AL and 58.1 months for patients with non-AL. For patients with AL management involved chemotherapy targeted toward monoclonal plasma cells, lymphocytes or both, and for patients with non-AL no specific amyloid treatment was administered. AL is a rare complication of CLL, but in this elderly population of patients non-AL is nearly as common. Distinguishing between these two groups is essential since patients with non-AL amyloidosis have better outcomes and they do not require cytotoxic chemotherapy to treat their amyloidosis.

摘要

为了阐明慢性淋巴细胞白血病(CLL)和淀粉样变性患者的表现和病程。我们检索了梅奥诊所 1974 年 1 月至 2012 年 10 月期间收治的淀粉样变性和 CLL 患者的梅奥数据库。我们对这些患者的病历进行了分析。在确定的 33 名患者中,20 名(61%)被诊断为 AL,13 名(39%)为非 AL。只有 4 名患者的免疫球蛋白轻链淀粉样变性(AL)可完全归因于 CLL 克隆;另有 6 名患者同时存在浆细胞克隆和与 CLL 克隆共享同一轻链的克隆。AL 患者的中位总生存期为 15.6 个月,而非 AL 患者的中位总生存期为 58.1 个月。AL 患者的治疗方案包括针对单克隆浆细胞、淋巴细胞或两者的化疗,而非 AL 患者未给予特定的淀粉样变性治疗。AL 是 CLL 的罕见并发症,但在这一年龄较大的患者人群中,非 AL 几乎同样常见。区分这两组至关重要,因为非 AL 淀粉样变性患者的预后更好,且他们不需要细胞毒性化疗来治疗淀粉样变性。

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