Chang Hong, Qi Xiaoying, Yeung Joanna, Reece Donna, Xu Wei, Patterson Bruce
Department of Laboratory Hematology, University Health Network, Toronto, Canada.
Leuk Res. 2009 Feb;33(2):259-62. doi: 10.1016/j.leukres.2008.06.027. Epub 2008 Aug 3.
Plasma cell leukemia (PCL) is a rare form of plasma cell malignancy, few large series reported underlying genetic abnormalities. We systematically evaluated the genomic aberrations in 41 PCL patients by combining fluorescence in situ hybridization with cytoplasmic light chain immunofluorescence and correlated with their clinical outcome. The genomic aberrations in the 15 primary PCL (pPCL) and 26 secondary PCL (sPCL) were compared with 220 newly diagnosed multiple myeloma (MM) patients. There was no significant difference in the prevalence of genetic abnormalities in pPCL and sPCL but del(13q), t(4;14), 1q21 amplification and del(1p21) were more common in PCL than MM. Patients with pPCL had higher creatinine and beta(2)-microglobulin levels and tended to have a longer overall survival than patients with sPCL. In univariant analysis, PCL patients with t(4;14) (p=0.006) and del(1p21) (p=0.003) had shorter overall survivals. In multivariant analysis adjusting for all tested genetic factors as well as clinico-biologically relevant factors including C-reactive protein, calcium and beta(2)-microglobulin, t(4;14) remained a significant predictor for adverse overall survival in PCL (p=0.008).
浆细胞白血病（PCL）是一种罕见的浆细胞恶性肿瘤，很少有大型系列报道其潜在的基因异常。我们通过将荧光原位杂交与细胞质轻链免疫荧光相结合，系统地评估了41例PCL患者的基因组畸变，并将其与临床结果相关联。将15例原发性PCL（pPCL）和26例继发性PCL（sPCL）的基因组畸变与220例新诊断的多发性骨髓瘤（MM）患者进行了比较。pPCL和sPCL中基因异常的发生率没有显著差异，但del(13q)、t(4;14)、1q21扩增和del(1p21)在PCL中比MM更常见。pPCL患者的肌酐和β2-微球蛋白水平较高，总体生存期往往比sPCL患者更长。在单变量分析中，伴有t(4;14)（p=0.006）和del(1p21)（p=0.003）的PCL患者总体生存期较短。在多变量分析中，对所有检测的基因因素以及包括C反应蛋白、钙和β2-微球蛋白在内的临床生物学相关因素进行校正后，t(4;14)仍然是PCL患者总体生存不良的一个重要预测因素（p=0.008）。
