Thomas Xavier, Le Quoc-Hung
Service d'Hématologie Clinique, Hôpital Edouard Herriot, Hospices Civils de Lyon, 69437 Lyon Cedex 03, France.
Hematology. 2008 Oct;13(5):293-302. doi: 10.1179/102453308X343374.
Central nervous system (CNS) involvement is identified at the time of diagnosis in less than 10% of adult acute lymphoblastic leukemia (ALL). These patients can attain long-term disease-free survival. CNS disease at presentation does not appear to be an independent poor prognostic factor. Because of the difficulty in treating CNS leukemia, innovative treatments and alternative delivery techniques are needed. The outcome in such patients is a reflection of an aggressive systemic and CNS-directed therapy. However, CNS toxicity represents the dose-limiting side effect of treatment. With effective CNS prophylaxis including intrathecal chemotherapy, high-dose systemic administration of certain agents and cranial irradiation, most adults with ALL without CNS disease at diagnosis may remain free of CNS leukemia. Leukemic relapse remains a major therapeutic problem and CNS involvement at the time of relapse occurs in 1-15% of cases. Adult ALL with CNS recurrence remains of poor prognosis and is generally associated with a systemic relapse.
在成年急性淋巴细胞白血病（ALL）患者中，诊断时中枢神经系统（CNS）受累的比例不到10%。这些患者能够实现长期无病生存。初诊时的中枢神经系统疾病似乎并非独立的不良预后因素。由于治疗中枢神经系统白血病存在困难，因此需要创新的治疗方法和替代给药技术。这类患者的治疗结果反映了积极的全身治疗和针对中枢神经系统的治疗。然而，中枢神经系统毒性是治疗的剂量限制性副作用。通过有效的中枢神经系统预防措施，包括鞘内化疗、某些药物的大剂量全身给药和颅脑照射，大多数诊断时无中枢神经系统疾病的成年ALL患者可能不会发生中枢神经系统白血病。白血病复发仍然是一个主要的治疗问题，复发时中枢神经系统受累的情况在1%至15%的病例中出现。伴有中枢神经系统复发的成年ALL预后仍然很差，通常与全身复发相关。
