退化细胞ZAP-70在慢性淋巴细胞白血病的表达及预后影响

  目的  探讨退化细胞、ZAP-70在慢性淋巴细胞白血病（CLL）患者外周血中的表达及其对预后的影响。  方法  利用光学显微镜观察退化细胞在92例CLL患者外周血涂片的检出率; 采用流式细胞术检测外周血淋巴细胞ZAP-70的阳性率。分析二者之间的相关性, 并观察这二项指标与疾病进展的关系。  结果  在92例CLL患者中, Binet A期28例（30.5%）, Binet B期44例（47.8%）, Binet C期20例（21.7%）。外周血涂片退化细胞百分比的中位数为19%（3%~48%）。取≥20%为阳性表达, 则退化细胞阳性56例（60.9%）; ZAP-70阳性32例（34.8%）。双因素直线相关分析提示二者呈负相关。退化细胞阴性患者临床预后差, 较阳性患者的8年无进展生存率（PFS）下降（59.4% vs. 86.0%, P < 0.05）, ZAP-70阳性患者较阴性患者的8年PFS也下降（57.8% vs. 79.5%, P < 0.05）; 退化细胞阴性伴ZAP-70阳性患者PFS更低（27.8%, P < 0.05）。  结论  退化细胞阴性和ZAP-70阳性提示CLL预后不良且二者密切相关。 

Expression and Prognostic Significance of Smudge Cells or ZAP-70 in Chronic Lymphocytic Leukemia

  Objective  To study the expression and prognostic significance of the percentage of smudge cells or ZAP-70 in chronic lymphocytic leukemia (CLL).  Methods  The percentage of smudge cells was observed on routine blood smears in a cohort of 92 CLL patients. ZAP-70 expression was determined by flow cytometry. The relationship of CLL progression with the smudge cells or ZAP-70 was analyzed.  Results  Among the 92 patients, 28 (30.5%) were Binet A, 44 (47.8%) were Binet B, and 20 (21.7%) were Binet C. The percentage of smudge cells ranged from 3% to 48% (median=19%). When the cutoff value was 20 %, 56 (60.9%) of the cases were positive for smudge cells, and 32 (34.8%) were positive for ZAP-70. A high ZAP-70 expression was found to be correlated with a low percentage of smudge cells. Patients with < 20% smudge cells (according to ROC analysis) were characterized by an unfavorable clinical course, i.e., significantly shorter progress-free survival, than those with ≥ 20% smudge cells (59.4% vs. 86%, P < 0.05). Patients with positive ZAP-70 expression had a significantly shorter progress-free survival than those with negative ZAP-70 expression (57.8% vs. 79.5%, P < 0.05). Combined analysis revealed a shorter progress-free survival in patients with < 20% smudge cells and positive ZAP-70 expression (27.8%, P < 0.05) than in those with < 20% smudge cells and negative ZAP-70 expression.  Conclusion  There is a close correlation between a smudge cell-negative status and positive ZAP-70 expression, which predicts unfavorable CLL prognosis.