Peijing Qi, Yan Xu, Yafei Wang, Dehui Zou, Zengjun Li, Junyuan Qi, Yaozhong Zhao, Lugui Qiu
Blood Disease Hospital and Institute of Hematology, Chinese Academy of Medical Science, Tianjin, China.
Acta Haematol. 2009;121(1):47-51. doi: 10.1159/000210555. Epub 2009 Apr 1.
BACKGROUND/AIMS: The study was undertaken to understand the characteristic of plasma cell leukemia (PCL) in China.
We reviewed and compared medical data of 22 primary PCL, 9 secondary PCL and 461 multiple myeloma (MM) diagnosed at our institute from January 2000 to January 2008.
The median onset ages of primary and secondary PCL patients were 49.5 and 56 years, respectively. PCL patients presented with a low peripheral blood plasmacytosis, extensive involvement of visceral organs and poor residual bone marrow function, without severe renal insufficiency. Seventeen of twenty-one PCL patients had abnormal karyotypes, mostly complex and hypodiploid or pseudodiploid. The deletion of 13q was frequent in primary and secondary PCL (57.1 and 42.9%, respectively). There was a significant difference in survival among primary PCL, secondary PCL and MM (median 14, 2 and 37 months, respectively, p = 0.0000).
Overall, primary and secondary PCL are different disorders with distinct natural histories and survival.
背景/目的:开展本研究以了解中国浆细胞白血病(PCL)的特征。
我们回顾并比较了2000年1月至2008年1月在我院诊断的22例原发性PCL、9例继发性PCL和461例多发性骨髓瘤(MM)的医学数据。
原发性和继发性PCL患者的中位发病年龄分别为49.5岁和56岁。PCL患者外周血浆细胞增多症程度低,内脏器官广泛受累,残余骨髓功能差,无严重肾功能不全。21例PCL患者中有17例核型异常,大多为复杂核型及亚二倍体或假二倍体。13q缺失在原发性和继发性PCL中均较为常见(分别为57.1%和42.9%)。原发性PCL、继发性PCL和MM的生存期存在显著差异(中位生存期分别为14个月、2个月和37个月,p = 0.0000)。
总体而言,原发性和继发性PCL是不同的疾病,具有不同的自然病程和生存期。