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. 2017 Jan 14;38(1):17-21.
doi: 10.3760/cma.j.issn.0253-2727.2017.01.004.

[Clinical features and prognosis in CD10(-) pre-B acute lymphoblastic leukemia]

[Article in Chinese]
X Y Gong  1 , Y Wang, B C Liu, H Wei, C L Zhou, D Lin, K Q Liu, S N Wei, B F Gong, G J Zhang, Y T Liu, X L Zhao, Y Li, R X Gu, S W Qiu, Y C Mi, J X Wang
Affiliations

[Clinical features and prognosis in CD10(-) pre-B acute lymphoblastic leukemia]

[Article in Chinese]
X Y Gong et al. Zhonghua Xue Ye Xue Za Zhi. .

Abstract
in English, Chinese

Objective: To analyze the clinical features and prognosis of acute lymphoblastic leukemia patients with immunophenotype of CD10(-)pre-B (CD10(-) pre B-ALL) . Methods: 6 adult cases with CD10(-) pre B-ALL immunophenotypes were analyzed retrospectively, related literatures were reviewed to clarify these kind of patients' clinical features and prognosis. Results: CD10(-) pre B-ALL occurred in 1.5% of ALL, 1.8% of B-ALL and 11.5% of pre B-ALL respectively. All the 6 patients were male with the median age as 33.5 years old, the median white blood cells was 101.78×10(9)/L, MLL-AF4 fusion transcripts were evident in all cases. Complete remission (CR) was achieved in 5 patients after first induction chemotherapy, 1 patient failed to respond to induction therapy, and got CR after 3 courses of chemotherapy. 2 patients underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) in CR(1), 1 patient relapsed in the short term and underwent allo-HSCT in CR(2). 1 patient was still waiting for allo-HSCT. Of the 2 patients who didn't receive transplantation, 1 died following a relapse, the other remained to be in CR. Conclusions: CD10(-) pre B-ALL was a rare but distinct subtype in adult ALL characterized by male dominance, high onset white blood cells and MLL rearrangement rate. Conventional chemotherapy produced a high response rate but more likely relapse, allo-HSCT may have the potential to improve the prognosis of these patients.

目的：分析CD10阴性的前B急性淋巴细胞白血病（CD10(-)pre B-ALL）患者的临床特征和预后。 方法：对6例成人CD10(-) pre B-ALL患者的临床和实验室资料进行回顾性分析，结合文献复习明确该类型患者的临床特征及预后。 结果： CD10(-)pre B-ALL占ALL的1.5%（6/409），占B-ALL的1.8%（6/343），占pre B-ALL的11.5%（6/52）。6例患者均为男性，中位年龄为33.5岁，起病时中位WBC为101.78×10(9)/L，所有患者均伴有MLL-AF4融合基因表达。5例患者经1个疗程诱导化疗即获得完全缓解（CR），1例患者经3个疗程化疗后才获得CR。2例患者在CR(1)期行异基因造血干细胞移植（allo-HSCT），1例患者CR后短期内即复发，在CR(2)期行allo-HSCT。1例患者正在等待移植。2例未移植患者1例复发死亡，1例尚处于缓解状态。 结论： CD10(-)pre B-ALL是一类具有独特临床特征的成人ALL亚型，发生率较低，常见于男性，起病时白细胞水平较高，MLL-AF4融合基因表达率高，常规化疗具有较高的缓解率，但易复发，allo-HSCT有可能改善其预后。.

Keywords: Gene rearrangement, MLL; Leukemia, lymphoid; Neprilysin; Prognosis.

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