淋巴母细胞淋巴瘤/白血病临床病理、免疫表型及预后相关性研究

目的 探讨淋巴母细胞淋巴瘤/急性淋巴母细胞白血病(LBL/ALL)临床病理、免疫组织化学特征并行预后相关性分析.方法 回顾性研究153例LBL/ALL患者的临床病理资料,根据临床和随访结果,对其预后与免疫表型、Ann Arbor分期、纵隔肿块、骨髓受累、肝脾肿大、Ki-67指数以及患者年龄、性别多因素进行相关性研究.结果 TdT和CIY99阳性率分别为79.1%(121/153例)和96.3%(131/136例).按免疫表型分为T-、B-LBI/ALL及未定类.(1)T-LBL/ALL占69.3%(106/153例),男75例,女31例,中位年龄17.5(2～68)岁,92例(86.8%)表现为外周淋巴结肿大,其中59例(55.7%)伴有纵隔肿块,91例(85.8%)为Ⅲ～进展期.1年与5年生存率分别为36.1%和8.1%,年龄大于25岁(P=0.049)、Ⅲ～Ⅳ期(P=0.001)是T-LBL/ALL预后不良的指征.(2)B-LBL/ALL占19.O%(29/153例),男18例,女11例,中位年龄14岁(9个月～75岁),17例(58.6%)表现为外周淋巴结肿大,其中13例(44.8%)有骨髓或外周血受累,5例(17.2%)伴有纵隔病变,21例病变处于Ⅲ～Ⅳ期.1年与5年生存率分别为53.3%和36.7%.(3)未定类组为11.7%(18/153例),所检抗体如CD3ε/CD3、CD45RO、CD79a、CD20、髓过氧化物酶(MPO)、CD5、CD56、cyclin D1、CK、神经元特异性烯醇化酶(NSE)、嗜铬粒素A(CgA)和突触素(Syn)均阴性.男13例,女5例,中位年龄15.5(4～53)岁,原发淋巴结病变15例(83.3%),7例(38.9%)有纵隔病变.(4)与B-LBL/ALL比较,T-LBI/ALL患者伴有纵隔肿块较为常见(P=0.0003),中位生存期短分别为(15.0±7.0)个月和(6.0±1.1)个月],但差异无统计学意义(P=0.07).结论 TdT与CD99是前驱淋巴细胞重要的特异性标志物;T-LBL/ALL多见于青少年男性,常伴有纵隔占位及浅表或多处淋巴结肿大;B-LBL/ALL易累及骨髓或外周血;T-LBL/ALL预后较差,年龄和临床分期是影响T-LBL/ALL患者生存的重要预后参数.

Lymphoblastic lymphoma and acute lymphoblastic leukemia: a clinicopathologic, immunophenotypic and prognostic study in 153 Chinese patients

Objective To study the clinicopathologic features, immunohistochemical findings and prognosis of precursor lymphoblastic lymphoma/acute lymphoblastic leukemia (LBL/ALL). Methods One hundred and fifty-three cases of LBL/ALL were retrospectively analyzed. Immunohistochemical study was carried out The pathologic findings were correlated with Ann Arbor tumor stage, Ki-67 index, other clinical parameters (including mediastinum/bone marrow involvement, hepato-splenomegaly, age and gender of the patients) and the survival data. Results Staining for TdT and CD99 was positive in 79. 1% (121/153 cases) and 96. 3% (131/136 cases) , respectively. The cases were categorized into three groups according to the immunohistochemical findings,as follows: precursor T-cell, precursor B-cell and undefined. T-LBI/ ALL accounted for 69. 3% ( 106/153 cases) of all of the cases. The male-to-female ratio was 2. 4: 1 (including 75 males and 31 females). The median age at diagnosis was 17. 5 years (ranged from 2 years to 68 years) . Ninety-two patients ( 86. 8% ) presented with peripheral lymphadenopathy and 59 of them (55. 7%) had mediastinal masses. Ninety-one cases (85. 8%) were in stage Ⅲ or Ⅳ at diagnosis. The 1-year and 5-year survival rates in patients with T-LBL/ALL were 36. 1% and 8. 1% , respectively. Patients older than 25 years and those presented in stage Ⅲ or Ⅳ suggested a poor prognosis (P = 0. 049 and 0. 001, respectively). On the other hand, 29 of the 153 cases (19. 0% ) belonged to B-LBL/ALL. The median age of the patients was 14 years ( ranged from 9 months to 75 years) . The male-to-female ratio was 1. 6:1 (including 18 males and 11 females) . Seventeen patients ( 58. 6%) presented with peripheral lymphadenopathy and 13 of them (44. 8%) had involvement of bone marrow or peripheral blood. Mediastinal involvement was found only in 5 cases (17. 2% ). Twenty-one patients (72. 4% ) were in stage Ⅲ or Ⅳ at diagnosis. The 1-year and 5-year survival rates were 53. 3% and 36. 7% , respectively. The remaining 11. 7% cases (18/153 cases) were categorized as undefined type, with a negative staining for the following immuno-markers including; CD3ε/CD3, CD45RO, CD79a, CD20, MPO, CD5,CD56,cyclin Dl, cytokeratin, neuron-specific enolase, chromogranin A and synaptophysin. The median age of the patients was 15. 5 years (ranged from 4 to 53 years). The male-to-female ratio was 2. 6:1 (including 13 males and 5 females). The percentage of T-LBL/ALL patients with mediastinal masses were significantly higher than that of B-LBL/ALL cases (P = 0. 0003). There was no significant difference in prognostic parameters of T-LBL/ALL and B-LBL/ALL (P =0. 07). The difference in median survival time however was statistically significant (6. 0 months ± 1. 1 months versus 15. 0 months ± 7. 0 months) . Conclusions Both TdT and CD99 are useful markers for the diagnosis of precursor lymphoblastic malignancy. T-LBL/ALL predominantly affects children or adolescent males and frequently presents with lymphadenopathy and mediastinal masses, whereas B-LBL/ALL are often accompanied by bone marrow and peripheral blood involvement In general, T-LBL/ALL carries a poor prognosis. The prognostic criteria include age of older than 25 years and a classification of stage Ⅲ or Ⅳ disease.