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.