Cesana Clara, Miqueleiz Sara, Bernuzzi Patrizia, Tresoldi Elisabetta, Rossi Valentina, D'avanzo Giovanna, Filippini Davide, Morra Enrica
Department of Hematology, Bone Marrow Transplantation Centre, Niguarda Ca' Granda Hospital, Milan, Italy.
Semin Oncol. 2003 Apr;30(2):231-5. doi: 10.1053/sonc.2003.50056.
Factors predicting evolution to symptomatic disease were investigated in 27 patients with smouldering Waldenstrom's macroglobulinemia (SWM) among 172 patients with Waldenstom's macroglobulinemia (WM), selected on the basis of the following criteria: (1) IgM paraprotein > 3 g/dL, and/or (2) bone marrow (BM) lymphoplasmacytoid (LPC) infiltration >or= 30%, and/or (3) diffuse infiltration pattern on BM biopsy, and (4) no treatment requirement for at least 12 months. Cumulative probability of survival was calculated by means of Kaplan-Meier. The Mantel and Haenszel test and multivariate Cox model were used to identify possible predictors for evolution. At a median follow-up of 79 months (range, 14 to 204), 11 patients (40.7%) showed progression to symptomatic disease, with the median interval from diagnosis being 46 months (range, 12 to 154). Event-free survival (EFS) at 5 and 10 years was 65% (95% confidence interval [CI], 45% to 85%) and 53% (95% CI, 31% to 75%), respectively. At multivariate analysis, paraprotein > 3 g/dL (hazard ratio [HR], 15.1; 95% CI, 3.01 to 75.64; P <.0009) and hemoglobin <or= 12.5 g/dL (HR, 3.75; 95% CI, 1.05 to 13.34; P <.042) independently predicted transformation into symptomatic disease (P <.0006). Neither BM findings nor other laboratory parameter was associated with overt WM development. In conclusion, in SWM monoclonal IgM levels > 3 g/dL and hemoglobin levels <or= 12.5 gr/dL are likely to predict SWM transformation into active disease requiring treatment.
在172例华氏巨球蛋白血症（WM）患者中，根据以下标准选择了27例冒烟型华氏巨球蛋白血症（SWM）患者，研究了预测向有症状疾病进展的因素：（1）IgM副蛋白>3 g/dL，和/或（2）骨髓（BM）淋巴浆细胞样（LPC）浸润≥30%，和/或（3）BM活检弥漫性浸润模式，以及（4）至少12个月无需治疗。通过Kaplan-Meier法计算累积生存概率。使用Mantel和Haenszel检验以及多变量Cox模型来确定进展的可能预测因素。中位随访79个月（范围14至204个月）时，11例患者（40.7%）出现向有症状疾病的进展，从诊断开始的中位间隔为46个月（范围12至154个月）。5年和10年的无事件生存率（EFS）分别为65%（95%置信区间[CI]，45%至85%）和53%（95%CI，31%至75%）。多变量分析时，副蛋白>3 g/dL（风险比[HR]，15.1；95%CI，3.01至75.64；P<.0009）和血红蛋白≤12.5 g/dL（HR，3.75；95%CI，1.05至13.34；P<.042）独立预测向有症状疾病的转化（P<.0006）。BM检查结果和其他实验室参数均与明显的WM发展无关。总之，在SWM中，单克隆IgM水平>3 g/dL和血红蛋白水平≤12.5 gr/dL可能预测SWM转化为需要治疗的活动性疾病。
