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[CD10(-)前B细胞急性淋巴细胞白血病的临床特征与预后]

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

作者信息

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

机构信息

Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.

出版信息

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

Abstract

To analyze the clinical features and prognosis of acute lymphoblastic leukemia patients with immunophenotype of CD10(-)pre-B (CD10(-) pre B-ALL) . 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. 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. 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.

摘要

分析CD10(-)前B细胞型急性淋巴细胞白血病(CD10(-) pre B-ALL)患者的临床特征及预后。回顾性分析6例具有CD10(-) pre B-ALL免疫表型的成人病例,并复习相关文献以明确此类患者的临床特征及预后。CD10(-) pre B-ALL分别占所有急性淋巴细胞白血病(ALL)的1.5%、B细胞型ALL的1.8%和前B细胞型ALL的11.5%。6例患者均为男性,中位年龄33.5岁,中位白细胞计数为101.78×10⁹/L,所有病例均检测到MLL-AF4融合转录本。5例患者首次诱导化疗后达到完全缓解(CR),1例诱导治疗无效,3个疗程化疗后达到CR。CR(1)期2例患者接受了异基因造血干细胞移植(allo-HSCT),1例短期内复发,CR(2)期接受allo-HSCT。1例患者仍在等待allo-HSCT。2例未接受移植的患者中,1例复发后死亡,另1例仍处于CR状态。CD10(-) pre B-ALL是成人ALL中一种罕见但独特的亚型,以男性为主、发病时白细胞计数高及MLL重排率高为特征。传统化疗缓解率高但复发可能性大,allo-HSCT可能有改善这些患者预后的潜力。

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