华氏巨球蛋白血症患者向弥漫性大 B 细胞淋巴瘤的组织学转化。
Histological transformation to diffuse large B-cell lymphoma in patients with Waldenström macroglobulinemia.
机构信息
Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusettts.
出版信息
Am J Hematol. 2016 Oct;91(10):1032-5. doi: 10.1002/ajh.24477. Epub 2016 Jul 27.
Histological transformation to diffuse large B-cell lymphoma (DLBCL) rarely occurs in patients with Waldenström Macroglobulinemia (WM). We identified 20 patients out of a cohort of 1,466 WM patients who experienced histologic transformation. The 5, 10, and 15-year cumulative incidence rates were 1, 2.4, and 3.8%, respectively. Approximately half of the patients were naive to nucleoside analogues, and a quarter were previously untreated for WM at the time of transformation. More than 80% of patients presented with extranodal involvement, 65% with high IPI scores. DLBCL cells did not express CD10 but expressed BCL6 and BCL2. All patients were treated with chemoimmunotherapy. The median survival from histological transformation was 2.7 years. The median overall survival was shorter for transformed patients versus those who did not transform (estimated 9 vs. 16 years; P = 0.09). Histological transformation to DLBCL is rare, and is associated with inferior survival in WM. Am. J. Hematol. 91:1032-1035, 2016. © 2016 Wiley Periodicals, Inc.
华氏巨球蛋白血症（WM）患者中很少发生弥漫性大 B 细胞淋巴瘤（DLBCL）的组织学转化。我们在 1466 例 WM 患者队列中发现了 20 例发生组织学转化的患者。5、10 和 15 年的累积发生率分别为 1%、2.4%和 3.8%。大约一半的患者对核苷类似物无反应，四分之一的患者在转化时未经 WM 治疗。超过 80%的患者有结外受累，65%的患者 IPI 评分较高。DLBCL 细胞不表达 CD10，但表达 BCL6 和 BCL2。所有患者均接受化疗免疫治疗。从组织学转化到 DLBCL 的中位生存时间为 2.7 年。与未转化的患者相比，转化患者的总生存期更短（估计分别为 9 年和 16 年；P=0.09）。WM 中 DLBCL 的组织学转化罕见，与生存不良相关。Am. J. Hematol. 91:1032-1035, 2016. © 2016 Wiley Periodicals, Inc.
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