Tsushima Takafumi, Terao Toshiki, Narita Kentaro, Fukumoto Ami, Ikeda Daisuke, Kamura Yuya, Kuzume Ayumi, Tabata Rikako, Miura Daisuke, Takeuchi Masami, Matsue Kosei
Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, Kamogawa, Japan.
Am J Clin Pathol. 2023 Aug 1;160(2):157-163. doi: 10.1093/ajcp/aqad013.
To demonstrate the clinical features and prognostic impact of cyclin D1 positivity in patients with amyloid light chain amyloidosis (AL).
We consecutively included 71 patients diagnosed with AL with cyclin D1 positivity between February 2008 and January 2022. t(11;14) was examined through interphase fluorescence in situ hybridization using bone marrow cells.
The median age of the patients was 73 years, and 53.5% were male. The underlying diseases included symptomatic multiple myeloma, smoldering multiple myeloma, Waldenström macroglobulinemia, and monoclonal gammopathy of undetermined significance, representing 33.8%, 26.8%, 2.8%, and 36.6%, respectively. The prevalence of cyclin D1 and t(11;14) was 38.0% and 34.7%, respectively. Higher frequency of light chain paraprotein type was seen in cyclin D1-positive patients with AL than in cyclin D1-negative patients (70.4% vs 18.2%). The median overall survival (OS) of patients with AL with and without cyclin D1 expression was 18.9 months and 73.1 months, respectively (P = .019). Early death occurred in 44.4% of cyclin D1-positive patients and 31.8% of cyclin D1-negative patients. Moreover, 83.3% of cyclin D1-positive patients and 21.4% of cyclin D1-negative patients died of cardiac causes.
Cyclin D1 immunohistochemistry accurately identified patients with t(11;14). Cyclin D1-positive patients had significantly inferior OS compared with cyclin D1-negative patients.
阐述细胞周期蛋白D1阳性在淀粉样轻链淀粉样变性（AL）患者中的临床特征及预后影响。
我们连续纳入了2008年2月至2022年1月期间诊断为AL且细胞周期蛋白D1阳性的71例患者。使用骨髓细胞通过间期荧光原位杂交检测t(11;14)。
患者的中位年龄为73岁，53.5%为男性。基础疾病包括有症状的多发性骨髓瘤、冒烟型多发性骨髓瘤、华氏巨球蛋白血症和意义未明的单克隆丙种球蛋白病，分别占33.8%、26.8%、2.8%和36.6%。细胞周期蛋白D1和t(11;14)的患病率分别为38.0%和34.7%。AL细胞周期蛋白D1阳性患者的轻链副蛋白类型频率高于细胞周期蛋白D1阴性患者（70.4%对18.2%）。有和没有细胞周期蛋白D1表达的AL患者的中位总生存期（OS）分别为18.9个月和73.1个月（P = 0.019）。44.4%的细胞周期蛋白D1阳性患者和31.8%的细胞周期蛋白D1阴性患者早期死亡。此外，83.3%的细胞周期蛋白D1阳性患者和21.4%的细胞周期蛋白D1阴性患者死于心脏原因。
细胞周期蛋白D1免疫组化准确识别出t(11;14)患者。与细胞周期蛋白D1阴性患者相比，细胞周期蛋白D1阳性患者的OS明显较差。
