Superior outcome of pediatric acute myeloid leukemia patients with orbital and CNS myeloid sarcoma: A report from the Children's Oncology Group
Superior outcome of pediatric acute myeloid leukemia patients with orbital and CNS myeloid sarcoma: A report from the Children's Oncology Group
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DOI：
10.1002/pbc.23201
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发表时间：
2012-04-01
影响因子：
3.2
通讯作者：
Woods, William G.
中科院分区：
文献类型：
--
作者：
Johnston, Donna L.;Alonzo, Todd A.;Woods, William G.
髓外白血病（EML）常见于儿童急性髓性白血病（AML），发生于脑脊液（CSF）中的白血病细胞或实体瘤（髓样肉瘤）。MS对生存的影响尚不清楚。方法. CCG方案2861、2891、2941和2961中接受强化定时化疗治疗AML的患者根据是否存在EML（CSF白血病、CNS-MS、眼眶- MS或非CNS MS）进行分类。CSF白血病被分类为CNS 3（在具有母细胞的CSF中> 5 WBC），非CSF白血病被分类为CNS 1/ 2（在具有或不具有母细胞的CSF中< 5 WBC）。比较这些患者的特征和结局。结果在1,459例患者中，1,206例（82%）无EML，154例（11%）有CSF白血病，19例（1%）有CNS-MS，23例（2%）有眼眶-MS，57例（4%）为非中枢神经系统MS，眼眶MS和中枢神经系统MS的CR率明显高于非MS和非中枢神经系统MS（96%和95%对比78%和78%，P 0.034）。眼眶MS和CNS MS患者的总生存率显著高于非CNS MS患者（92%和73% vs. 38%，P < 0.001）、CNS 3患者（92%和73% vs. 51，P < 0.001）和CNS 1/ 2患者（92%和73% vs. 50%，P < 0.001）。眼眶MS患者的复发率明显较低。结论累及眼眶和中枢神经系统的MS患者的生存率显著高于非中枢神经系统MS、CSF白血病或无EML患者。儿科血液癌症2012; 58：519-524。(C)2011 Wiley Periodicals，Inc.
Background Extramedullary leukemia (EML) is common in pediatric acute myeloid leukemia (AML) and occurs as leukemia cells within the cerebrospinal fluid (CSF) or as a solid tumor (myeloid sarcomaMS). The effect of MS on survival is unknown. Methods. Patients on CCG protocols 2861, 2891, 2941, and 2961 being treated for AML with intensive-timing chemotherapy were classified for the presence of EML (CSF leukemia, CNS-MS, orbital- MS, or non-CNS MS). CSF leukemia was classified as CNS3 (> 5 WBC in the CSF with blasts) and non-CSF leukemia as CNS1/ 2 (< 5 WBC in the CSF with or without blasts). Characteristics and outcomes of these patients were compared. Results. Of the 1,459 total patients, 1,206 (82%) had no EML, 154 (11%) had CSF leukemia, 19 (1%) had CNS-MS, 23 (2%) had orbital-MS, and 57 (4%) had non-CNS MS. The CR rate was significantly higher in patients with orbital-MS and CNS-MS than in those with non-MS and non-CNS MS (96% and 95% vs. 78% and 78%, P 0.034). Patients with orbital-MS and CNS-MS had significantly higher overall survival than patients with non-CNS MS (92% and 73% vs. 38%, P < 0.001), CNS3 patients (92% and 73% vs. 51, P < 0.001), and CNS1/ 2 patients (92% and 73% vs. 50%, P < 0.001). Patients with orbitalMS had a significantly lower relapse rate. Conclusion. Patients with MS involving orbital and CNS sites had a significantly better survival than patients with non-CNS MS, with CSF leukemia, or with no EML. Pediatr Blood Cancer 2012; 58: 519-524. (C) 2011 Wiley Periodicals, Inc.