Abbott B L, Rubnitz J E, Tong X, Srivastava D K, Pui C-H, Ribeiro R C, Razzouk B I
St Jude Children's Research Hospital, University of Tennessee, Memphis, TN 38105-2794, USA.
Leukemia. 2003 Nov;17(11):2090-6. doi: 10.1038/sj.leu.2403131.
To determine the clinical significance of central nervous system (CNS) involvement at the time of diagnosis of pediatric acute myeloid leukemia (AML), we analyzed clinical features and outcomes of 290 patients treated consecutively on four institutional trials (AML80, AML83, AML87, and AML91). CNS status was classified as CNS1 (no blast cells in CSF; n=205), CNS2 (<5 WBC/mul CSF with blast cells; n=37), or CNS3 (>/=5 WBC/mul CSF with blast cells, or signs of CNS involvement; n=48). Patients with CNS3 status were significantly younger than others (P=0.016) and significantly more likely to have the favorable cytogenetic features t(9;11), t(8;21), or inv(16) (P<0.001). The CNS3 group had a significantly greater probability (+/-s.e.) of 5-year event-free survival (43.7+/-7.0%) than did the CNS1 (27.8+/-3.2%, P=0.015) and CNS2 (24.3+/-7.5%, P=0.032) groups. However, after adjustment for favorable genetic features, there was no significant difference in EFS between the CNS3 and the combined CNS1+CNS2 groups (P=0.075). In all, 10 of 151 patients treated on AML80 and AML83, but none of 139 treated on AML87 and AML91, had primary CNS relapse. CNS involvement had no adverse prognostic significance, and patients with CNS2 status had similar outcome to CNS1 patients in this large group of pediatric patients with AML, treated at a single institution.
为确定小儿急性髓系白血病（AML）诊断时中枢神经系统（CNS）受累的临床意义，我们分析了在四项机构试验（AML80、AML83、AML87和AML91）中连续治疗的290例患者的临床特征和预后。CNS状态分为CNS1（脑脊液中无原始细胞；n = 205）、CNS2（脑脊液中每微升白细胞计数<5且有原始细胞；n = 37）或CNS3（脑脊液中每微升白细胞计数≥5且有原始细胞，或有CNS受累迹象；n = 48）。CNS3状态的患者比其他患者明显年轻（P = 0.016），且更有可能具有有利的细胞遗传学特征t(9;11)、t(8;21)或inv(16)（P<0.001）。CNS3组的5年无事件生存率（43.7±7.0%）明显高于CNS1组（27.8±3.2%，P = 0.015）和CNS2组（24.3±7.5%，P = 0.032）。然而，在对有利的基因特征进行校正后，CNS3组与CNS1 + CNS2联合组的无事件生存率无显著差异（P = 0.075）。在AML80和AML83治疗的151例患者中，有10例发生原发性CNS复发，但在AML87和AML91治疗的139例患者中均无复发。在这组在单一机构接受治疗的大量小儿AML患者中，CNS受累没有不良预后意义，CNS2状态的患者与CNS1患者的预后相似。
