Kobayashi Ryoji, Tawa Akio, Hanada Ryoji, Horibe Keizo, Tsuchida Masahiro, Tsukimoto Ichiro
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Pediatr Blood Cancer. 2007 Apr;48(4):393-8. doi: 10.1002/pbc.20824.
Extramedullary infiltration (EMI) is an occasional clinical symptom in childhood acute myelogenous leukemia (AML), but there is considerable controversy regarding the prognostic significance of EMI in AML.
We evaluated the frequency and prognostic significance of EMI at diagnosis of AML in children.
Of 240 cases of de novo AML excluding children with Down syndrome and acute promyelocytic leukemia, 56 (23.3%) showed EMI at diagnosis. Patients with EMI had a higher initial WBC count and a higher proportion of M4/M5 morphological variants. The complete remission rate following induction chemotherapy was lower in patients with EMI. However, the overall survival and event-free survival did not differ between patients with and without EMI. A detailed analysis showed that patients with EMI with a WBC count at diagnosis of over 100 x 10(9)/L or infiltration into the central nervous system are likely to have a poor prognosis.
CNS leukemia and EMI together with a WBC count of >100 x 10(9)/L at diagnosis of AML are high risk factors for relapse, and alternative treatment approaches for patients with these characteristics should be explored.
髓外浸润(EMI)是儿童急性髓系白血病(AML)偶尔出现的临床症状,但关于EMI在AML中的预后意义存在相当大的争议。
我们评估了儿童AML诊断时EMI的发生率及其预后意义。
在240例排除唐氏综合征患儿和急性早幼粒细胞白血病的初发AML病例中,56例(23.3%)在诊断时有EMI。有EMI的患者初始白细胞计数较高,M4/M5形态学变异比例较高。诱导化疗后的完全缓解率在有EMI的患者中较低。然而,有或无EMI的患者总生存期和无事件生存期并无差异。详细分析显示,诊断时白细胞计数超过100×10⁹/L或浸润至中枢神经系统的有EMI的患者预后可能较差。
AML诊断时中枢神经系统白血病、EMI以及白细胞计数>100×10⁹/L是复发的高危因素,对于具有这些特征的患者应探索替代治疗方法。