Kriangkum Jitra, Taylor Brian J, Mant Michael J, Treon Steven P, Belch Andrew R, Pilarski Linda M
Department of Oncology, University of Alberta, Cross Cancer Institute, Edmonton, Canada.
Semin Oncol. 2003 Apr;30(2):132-5. doi: 10.1053/sonc.2003.50061.
The unique IgM VDJ sequence that characterizes the malignant clone in Waldenstrom's macroglobulinemia (WM), termed clonotypic, was identified for 12 WM patients. The majority of WM patients (92%) had a clonotypic IgM from the VH3 family, with predominantly long CDR3 regions, characteristic of those found in antigen-stimulated populations. Clonotypic IgM transcripts were detected in both blood and bone marrow (BM), clearly identifying a blood-borne compartment of WM. Abnormal numbers of CD20(+) B cells were usually detectable and expressed surface IgM. In most cases these cells also expressed surface IgD. Most WM patients lacked detectable CD138(+) plasma cells in either blood or BM. Longitudinal analysis suggests that phenotypic identification of B cells in blood of WM patients is insufficient for monitoring disease. Although serum IgM had decreased and clonotypic transcripts were very weak for one patient, the number of CD20(+) B cells increased dramatically. The lack of clonotypic transcripts suggests that the majority of these circulating B cells were polyclonal and were not part of the WM clone, indicating that monitoring of clonotypic IgM provides the most accurate identifier of WM cells.
在12例华氏巨球蛋白血症（WM）患者中鉴定出了独特的IgM VDJ序列，该序列可表征WM中的恶性克隆，称为克隆型。大多数WM患者（92%）具有来自VH3家族的克隆型IgM，其CDR3区域主要较长，这是在抗原刺激群体中发现的特征。在血液和骨髓（BM）中均检测到克隆型IgM转录本，明确确定了WM的血源区室。通常可检测到异常数量的CD20(+) B细胞，并表达表面IgM。在大多数情况下，这些细胞也表达表面IgD。大多数WM患者在血液或骨髓中均未检测到可检测到的CD138(+)浆细胞。纵向分析表明，WM患者血液中B细胞的表型鉴定不足以监测疾病。尽管一名患者的血清IgM有所下降且克隆型转录本非常微弱，但CD20(+) B细胞数量却急剧增加。缺乏克隆型转录本表明，这些循环B细胞中的大多数是多克隆的，并非WM克隆的一部分，这表明监测克隆型IgM可提供WM细胞最准确的标识符。
