[混合表型急性白血病患者的免疫表型特征及临床预后]。

Tie-Qiang Liu, Shan Huang, Xin-Yang Li, Bing-Xia Li, Bo Yao, Rui Zhang, Yi Wang, Zhi-Qing Liu, Kai-Xun Hu, Bo Cai, Chang-Lin Yu, Jian-Hui Qiao, Mei Guo
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引用次数: 0

摘要

目的:回顾性分析31例混合表型急性白血病(MPAL)患者的实验室检查结果及临床资料,总结并探讨基于免疫表型结果的MPAL各亚型的生物学特点、疗效及预后。方法:选取2013年7月至2019年1月在我院诊治的MPAL患者,分析初诊时细胞形态学、免疫表型、细胞遗传学、分子生物学(MICM)及血常规等数据。随访至最后一次出院。结果:31例患者中,男性19例,女性12例,中位年龄41(12 ~ 76)岁。免疫表型及EGIL评分结果显示,骨髓- t淋巴混合表型(骨髓- t组)16例,骨髓- b淋巴混合表型(骨髓- b组)9例,T-B淋巴混合表型(T-B组)5例,骨髓-T-B淋巴混合表型1例。不同亚型间比较,抗原表达特征以骨髓- b组HLA-DR阳性率和表达率最高,T-B组CD2阳性率显著高于骨髓- t组。同时,T-B组CD7和细胞质CD3的表达率高于骨髓- t组,cCD79a在骨髓- b组和T-B组均呈阳性。T-B组WBC中位数为81.92×109/L,显著高于其他两组(P-4占92.6%),骨髓b组WT1表达水平显著低于其他两组(p结论:WT1基因在MPAL患者中高表达,且基于免疫表型的MPAL各亚组均有其独特的抗原表达特征。与骨髓- t组和T-B组相比,骨髓- b组缓解率更高,预后更好。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Immunophenotypic Features and Clinical Prognosis of Patients with Mixed Phenotype Acute Leukemia].

Objective: To retrospectively analyze the laborotary test results and clinical data of 31 patients with mixed phenotype acute leukemia (MPAL) in order to summarize and discuss the biological characteristics, curative effect, and prognosis of each subtype of MPAL based on immunophenotype results.

Methods: MPAL patients diagnosed and treated in our hospital from July 2013 to January 2019 were selected to analyze the data of cell morphology, immunophenotyping, cytogenetics, molecular biology (MICM), and routine blood at initial diagnosis. Follow-up was carried out until the last discharge time.

Results: Among 31 patients, there were 19 males and 12 females, with a median age of 41(12-76) years old. According to the results of immunophenotyping and EGIL score, there were 16 cases of myeloid-T lymphoid mixed phenotype (myeloid-T group), 9 cases of myeloid-B lymphoid mixed phenotype (myeloid-B group), 5 cases of T-B lymphoid mixed phenotype (T-B group), and 1 case of myeloid-T-B lymphoid mixed phenotype. Compared between different subtypes, the antigen expression characteristics were the highest positive rate and expression rate of HLA-DR in myeloid-B group, and the positive rate of CD2 in T-B group was significantly higher than that in the myeloid-T group. Meanwhile, the expression rates of CD7 and cCD3 (cytoplasmic CD3) in T-B group were higher than those in myeloid-T group, and cCD79a was positive in all cases of myeloid-B group and T-B group. The median WBC of T-B group was 81.92×109/L, which was significantly higher than that of the other two groups (P<0.05). The quantitative results of WT1 were higher than 10-4 in 92.6% of the patients, and the WT1 expression level in myeloid-B group was significantly lower than the other two groups (P<0.01). Among the 9 patients with myeloid-B mixed phenotype, 5 cases showed BCR-ABL positive. Among 28 patients followed up, 21 cases achieved complete remission (CR), the median time to first obtain CR was 32.5(9-75) days, and the median follow-up time was 16 months (range from 21 days to 6 years). The CR rate and median overall survival (OS) time in myeloid-B group were 88.9% and 40 months, which were higher than the other two groups. The CR rate and 3-year OS rate in T-B group were relatively lower (50.0%, 0).

Conclusion: WT1 gene is highly expressed in patients with MPAL, and each subgroup of MPAL based on immuophenotype has its unique antigen expression characteristics. Compared with myeloid-T group and T-B group, myeloid-B group can acquire higher remission rate and have better prognosis.

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