Davey F R, Lawrence D, MacCallum J, Varney J, Hutchison R, Wurster-Hill D, Schiffer C, Sobol R E, Ciminelli N, Le Beau M
Department of Pathology, SUNY Health Science Center, Syracuse 13210.
Am J Hematol. 1992 Jul;40(3):183-91. doi: 10.1002/ajh.2830400306.
The CALGB prospectively studied 140 adult acute lymphoblastic leukemia (ALL) patients for cytogenetic abnormalities. Seven (5%) patients with adequate cytogenetic preparations had t(8;14)(q24;q32) or t(8;22)(q24;q11). Patients were compared with non-8q24 patients for clinical and laboratory characteristics, response to therapy, and survival. The median age of patients with translocations involving 8q24 (71% males) was 40 years. Forty-three percent had lymphadenopathy, 29% splenomegaly, and 29% hepatomegaly. None exhibited central nervous system (CNS), skin, or gum involvement. These features did not differ significantly from non-8q24 ALLs. Patients with 8q24 translocations had higher hemoglobins (11.5 vs. 9.8 g/dl; P = 0.04) and lower percentage of blasts in the peripheral blood (8.5% vs. 69%; P = 0.007). Although all seven were finally categorized as ALL-L3, a marked variation in the proportion of typical L3 blasts was observed that initially resulted in the diagnoses of ALL-L2 in three cases and prolymphocytic leukemia in one. In five of five patients, the blasts typed as B cells (SIg+ and CD19+). Complete remission rates for patients with 8q24 translocations were 43%, whereas they were 68% for non-8q24 ALLS (P = 0.22). Furthermore, patients with 8q24 abnormalities exhibited significantly shorter survival (4.8 vs. 18.4 mo; P less than 0.001). We conclude that ALL with translocations of 8q24 in adults shows a mature B-cell immunophenotype (SIg+), poor prognosis and morphology ranging from classical ALL-L3 to ALL with a subpopulation of L3 cells. Thus, the diagnosis of ALL-L3 should be made when blastic cells possess a mature B-cell immunophenotype (SIg+) and an 8q24 translocation, even though the number of L3 cells is low.
癌症和白血病研究组B(CALGB)对140例成人急性淋巴细胞白血病(ALL)患者的细胞遗传学异常进行了前瞻性研究。7例(5%)细胞遗传学准备充分的患者存在t(8;14)(q24;q32)或t(8;22)(q24;q11)。将这些患者与非8q24患者在临床和实验室特征、治疗反应及生存情况方面进行了比较。涉及8q24易位的患者中位年龄为40岁(71%为男性)。43%有淋巴结病,29%有脾肿大,29%有肝肿大。均未出现中枢神经系统(CNS)、皮肤或牙龈受累情况。这些特征与非8q24的ALL无显著差异。8q24易位的患者血红蛋白水平较高(11.5对9.8 g/dl;P = 0.04),外周血原始细胞百分比更低(8.5%对69%;P = 0.007)。尽管所有7例最终都被归类为ALL-L3,但观察到典型L3原始细胞比例存在明显差异,最初有3例被诊断为ALL-L2,1例被诊断为幼淋巴细胞白血病。5例患者中有5例原始细胞分型为B细胞(表面免疫球蛋白阳性和CD19阳性)。8q24易位患者的完全缓解率为43%,而非8q24的ALL患者为68%(P = 0.22)。此外,8q24异常的患者生存时间显著更短(4.8对18.4个月;P小于0.001)。我们得出结论,成人中伴有8q24易位的ALL表现出成熟B细胞免疫表型(表面免疫球蛋白阳性)、预后不良,形态学范围从经典的ALL-L3到伴有L3细胞亚群的ALL。因此,即使L3细胞数量较少,当原始细胞具有成熟B细胞免疫表型(表面免疫球蛋白阳性)且存在8q24易位时,也应诊断为ALL-L3。