儿童急性淋巴细胞白血病中髓系抗原表达的临床重要性

Clinical importance of myeloid-antigen expression in acute lymphoblastic leukemia of childhood.

作者信息

Wiersma S R, Ortega J, Sobel E, Weinberg K I

机构信息

Division of Hematology/Oncology, Childrens Hospital Los Angeles.

出版信息

N Engl J Med. 1991 Mar 21;324(12):800-8. doi: 10.1056/NEJM199103213241204.

DOI:10.1056/NEJM199103213241204

PMID:1997852

Abstract

BACKGROUND

Leukemic cells in 15 to 25 percent of patients with acute lymphoblastic leukemia (ALL) express myeloid antigens as well as lymphoid antigens (the latter reflecting B-cell or T-cell lineage). The relations of myeloid-antigen expression to other features of ALL and to prognosis have been controversial.

METHODS

We analyzed clinical and laboratory features present at diagnosis in 236 consecutive cases of ALL in children. Immunophenotyping, including single- and dual-fluorescence analyses, was used to classify leukemic cells as B or T lymphoblasts and also to identify myeloid-antigen expression--the simultaneous expression of lymphoid-associated antigens and at least one of three myeloid-associated antigens (CD33, CD13, and CD14) on cells classified as L1 or L2 according to the French-American-British system.

RESULTS

Forty-five of 185 patients with B-lineage ALL had myeloid-antigen expression, as did 8 of 41 patients with T-lineage ALL. In 10 patients, the lineage could not be determined. Myeloid-antigen expression was associated with L2 morphology (P less than 0.05), but it did not correlate with other prognostic features recognized previously. Multivariate analysis showed that myeloid-antigen expression was an important predictor of relapse in childhood ALL and the most significant prognostic factor statistically (P less than 0.0001). A white-cell count greater than or equal to 50 x 10(9) per liter at diagnosis was also an important and highly significant prognostic feature (P less than 0.001). After 40 months, the estimated disease-free survival for patients with ALL was 84 percent for those without myeloid-antigen expression and with a low white-cell count, 57 percent for those without myeloid-antigen expression and with a high white-cell count, 47 percent for those with myeloid-antigen expression and a low white-cell count, and 26 percent for those with myeloid-antigen expression and a high white-cell count (P less than 0.00001).

CONCLUSIONS

Myeloid-antigen expression is an important independent predictor of a poor response to chemotherapy in childhood ALL.

摘要

背景

在15%至25%的急性淋巴细胞白血病（ALL）患者中，白血病细胞同时表达髓系抗原和淋巴系抗原（后者反映B细胞或T细胞系）。髓系抗原表达与ALL的其他特征及预后的关系一直存在争议。

方法

我们分析了236例连续儿童ALL病例诊断时的临床和实验室特征。采用免疫表型分析，包括单荧光和双荧光分析，将白血病细胞分类为B或T淋巴母细胞，并确定髓系抗原表达情况，即在按照法美英系统分类为L1或L2的细胞上同时表达淋巴系相关抗原和三种髓系相关抗原（CD33、CD13和CD14）中的至少一种。

结果

185例B系ALL患者中有45例表达髓系抗原，41例T系ALL患者中有8例表达髓系抗原。10例患者的细胞系无法确定。髓系抗原表达与L2形态相关（P<0.05），但与先前确认的其他预后特征无关。多因素分析显示，髓系抗原表达是儿童ALL复发的重要预测指标，也是统计学上最显著的预后因素（P<0.0001）。诊断时白细胞计数≥50×10⁹/L也是一个重要且高度显著的预后特征（P<0.001）。40个月后，ALL患者中无髓系抗原表达且白细胞计数低者的预计无病生存率为84%，无髓系抗原表达且白细胞计数高者为57%，有髓系抗原表达且白细胞计数低者为47%，有髓系抗原表达且白细胞计数高者为26%（P<0.00001）。