儿童中枢神经系统急性淋巴细胞白血病诊治进展

儿童急性淋巴细胞白血病是可治愈性疾病,多数患儿经过系统化治疗可持续完全缓解,但仍有部分复发,其中中枢神经系统受累是导致患儿复发以及影响生存质量的重要原因之一。临床儿科医生对中枢神经系统急性淋巴细胞白血病的诊断、防治等相关进展应有所了解。     

小儿急性淋巴细胞白血病中枢神经系统白血病诊治现状

中枢神经系统白血病(CNSL)的防治是小儿急性淋巴细胞白血病(ALL)治疗的一部分。诊断时高白细胞计数、T细胞型及分子遗传学为t(4;11)和Ph 是CNS复发的危险因素,脑脊液不同检查结果的预后价值有待明确。头颅放疗已不用于标危ALL患儿,头颅放疗的预防剂量已减为12Gy,鞘内及全身化疗对CNSL的治疗有重要作用。部分小儿CNS复发经挽救治疗可以长期存活,早期CNS复发的患儿应在第2次CR期进行异基因骨髓移植。     

以肥胖为首发症状的复发性中枢神经系统白血病

目的分析罕见的以肥胖为首发症状的中枢神经系统白血病(CNSL)的诊断。方法 1例6岁4个月男孩,近3个月来进行性肥胖伴性格改变,以往患急性淋巴细胞白血病(ALL,普通B细胞,BCR-ABL阴性,中危),经化疗后持续缓解超过2年。根据临床表现做一系列的实验室和影像学检查进行诊断和鉴别诊断,并进行相关中英文文献复习。结果脑脊液(CSF)白细胞中度升高,以单个核细胞为主,伴少量可疑的幼稚淋巴细胞,葡萄糖和氯化物低,蛋白稍高,MRI示脑膜、大脑皮质、下丘脑和垂体柄以及桥脑等中线结构、颅神经根等信号异常。临床检查分析排除单纯肥胖、Cushing综合征等继发性肥胖和结核性脑膜炎;CSF流式细胞术检查发现65%的有核细胞符合ALL细胞的特征而诊断为CNSL复发,经化疗和头颅放疗后缓解。目前中、英文文献只检索到3篇相似病例的个案报道。结论肥胖和性格改变可以是CNSL的首发症状,但由于罕见,需注意与其他疾病鉴别,传统的CSF细胞形态学结合流式细胞术检查将提高CNSL诊断的可靠性。     

中枢神经系统白血病的诊治现状

采用现代治疗方案已使70%~80%小儿急性淋巴细胞白血病(ALL)达到长期无病生存即治愈。中枢神经系统白血病(CNSL)的预防治疗是小儿ALL治疗的组成部分。如果不进行系统的针对CNSL的预防治疗,整个疾病过程中CNSL发生率达70%。采取防治措施后CNSL发生率可降至5%以下。本文就CNSL若干     

流式细胞术在诊断儿童中枢神经系统白血病中的意义

目的 探讨流式细胞术(FCM)在诊断儿童中枢神经系统白血病(CNSL)中的意义.方法 分别采用FCM及细胞形态学(CC)检测急性淋巴细胞白血病(ALL)患儿脑脊液(CSF),对比这两种方法诊断儿童CNSL的敏感度.结果 脑脊液FCM检出67例异常表型细胞,检出率为20.36％;脑脊液CC检出6例异常细胞,检出阳性率1....     

脂质体阿糖胞苷治疗儿童中枢神经系统白血病的研究进展

急性白血病（AL）是儿童时期最常见的恶性肿瘤。近20年来,随着新的抗白血病药物的先后问世,联合化疗方案和其它治疗方法的不断改进和提高,儿童AL的远期疗效得到明显提高。采用现代治疗方案已使儿童急性淋巴细胞白血病（ALL）和急性髓系白血病（AML）的5年无病生存率（DFS）分别可达到70％-80％和50％。     

儿童急性淋巴细胞性白血病中枢神经系统浸润的防治

目的通过对中枢神经系统白血病(CNSL)的防治,预防和降低白血病的髓外复发机会,提高儿童急性淋巴细胞性白血病(ALL)的长期生存。 方法1999~2003年上海市新华医院对277例儿童ALL的诱导治疗期4~5次鞘内三联注射(甲氨蝶呤、阿糖胞苷、地塞米松),巩固期后采用大剂量甲氨蝶呤(HD-MTX)24h静脉连续滴注,进入维持后,每3个月1次,总共8~9次,以后改为鞘内注射3个月1次,直至化疗结束,对于超高危ALL患儿(白细胞计数>100×10⁹/L、Ph1染色体阳性)采用头颅照射加鞘内注射。 结果277例ALL患儿发生CNSL 9例,发生率为3.2%,9例CNSL中4例骨髓复发,5例存活,中位生存时间22.2个月。 结论CNSL的防治方法,明显降低了CNSL的发生率,使ALL患儿生存机会提高。发生CNSL的不利因素有高白细胞血症、T细胞性ALL、Ph1阳性染色体改变等。     

急性淋巴细胞白血病并中枢神经系统白血病的诊断与治疗

目的：探讨急性淋巴细胞白血病（ALL）并中枢神经系统白血病（CNSL）的诊断与治疗及影响发病和预后的因素。方法：对1990－1999年收治117例临床资料进行回顾分析。结果：CNSL发生距确诊ALL的中位数时间为8个月,高危型组发生率（54．85）明显高于标危型组（23．7％）。31例CNSL中以脑脊液（CSF）异常作出诊断远比临床症状多。CNSL治疗效果显示,大剂量氨甲喋呤＋三联鞘注＋四组（CR＋IT）相当。结论：为避免诊断假阳性造成的过度治疗,CSF仅有幼稚细胞而白细胞计数政党者诊断CNSL应慎重,HDMTX＋IT＋FC是治疗CNSL的有效措施。     

以中枢神经系统症状起病的儿童急性淋巴细胞白血病3例

目的 总结以单纯中枢神经系统症状起病的3例白血病患儿的临床特点,为临床诊治提供新的思路.方法 对3例以神经系统症状起病的急性淋巴细胞白血病患儿进行回顾及随访研究,总结其病例特点、治疗方案及预后,并结合既往文献提出其可能的发病机制.结果 3例患儿,男2例,女1例,起病年龄8~13岁.以单纯中枢神经系统异常表现为首发症状,伴有不突出的三系减少,未引起重视.多系统实验室检查指标异常,伴有血沉、铁蛋白、IL-6等炎症指标升高.脑脊液检查均正常,头颅MRI异常信号未给予化疗自行缓解.病初均被误诊,于病程3个月至1年2个月确诊为白血病.病例1已化疗2年半,未给予颅脑放疗,骨髓完全缓解;病例2放弃治疗;病例3给予诱导化疗后骨髓完全缓解,已给予治疗4个月.结论 以单纯中枢神经系统症状起病的急性白血病较为罕见、常被误诊.应重视病初伴有的轻度血液系统异常.3例中神经系统的异常,并非白血病细胞直接浸润所致,可能为副肿瘤综合征血管炎样表现.     

儿童急性淋巴细胞白血病 53例疗效分析

目前国外对小儿急性淋巴细胞白血病（ALL）的治疗进展较快,缓解率（CR）已达95％以上,5年以上无事故生存率（EFS）达70％～80％。我国经过多年临床实践,于1998年在山东荣城召开的全国小儿血液病会议期间,制定了一套符合国情的小儿血液病诊疗方案。我院按此方案治疗53例小儿ALL．现总结分析如下。     

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??Childhood acute lymphoblastic leukemia is the first disseminated cancer shown to be curable. Central nervous system leukemia ??CNSL?? is one of reasons that cause leukemia replase and is associated with a poor prognosis. Pediatricians should master the diagnostic techniques??modalities of prophylaxis and therapy of CNSL in patients with childhood acute lymphoblastic leukemia.     

CNS-3 status remains an independent adverse prognosis factor in children with acute lymphoblastic leukemia (ALL) treated without cranial irradiation: Results of EORTC Children Leukemia Group study 58951

AimTo evaluate the prognostic significance of initial central nervous system (CNS) involvement of children with acute lymphoblastic leukemia (ALL) enrolled in the EORTC 58951 trial.Patients and methodsFrom 1998 to 2008, 1930 ALL patients were included in the randomized EORTC 58951 trial. Overall treatment intensity was adjusted according to known prognostic factors including the level of minimal residual disease after induction treatment. CNS-directed therapy comprised four to 11 courses of i.v. methotrexate (5 g/m²), and 10 to 19 intrathecal chemotherapy injections, depending on risk group and CNS status. Cranial irradiation was omitted for all patients.ResultsThe overall 8-year event-free survival (EFS) and overall survival (OS) rates were 81.3% and 88.1%, respectively. In the CNS-1, TPL+, CNS-2, and CNS-3 groups, the 8-year EFS rates were 82.1%, 77.1%, 78.3%, and 57.4%, respectively. Multivariable analysis indicated that initial CNS-3 status, but not CNS-2 or TLP+, was an independent adverse predictor of outcome. The 8-year incidence of isolated CNS relapse was 1.7% and of isolated or combined CNS relapse it was 3.7%. NCI high-risk group, male sex, CNS-2 and CNS-3 status were independent predictors for a higher incidence of any CNS relapse.ConclusionsCNS-3 status remains associated with poor prognosis and requires intensification of both systemic and CNS-directed therapy.This trial was registered at https://clinicaltrials.gov/under/NCT00003728.     

儿童中枢神经系统白血病22例临床分析

目的分析22例急性白血病合并中枢神经系统白血病(CNSL)患儿的住院资料,为CNSL诊治提供参考。方法分析22例儿童CNSL的临床特征、实验室检查及预后。结果在303例儿童急性白血病中发生CNSL 22例,发生率7.3%,其中急性淋巴细胞白血病(ALL)18例,急性混合型白血病(MPAL)1例,急性髓细胞白血病(AML)3例;在18例ALL中,B-ALL 11例,T-ALL 7例,T-ALL合并CNSL发生率(22.6%)高于B-ALL(5.6%);CNSL常见临床表现有头痛、呕吐、惊厥及瘫痪等;CNSL预后差,死亡率高。结论 CNSL是急性白血病复发的主要原因之一,根据危险度来预防和治疗CNSL是关键。     

Relapse of precursor B-cell acute lymphoblastic leukemia as an isolated central nervous system mass lesion 9 years after initial diagnosis

Seven years after completion of chemotherapy for acute lymphoblastic leukemia, diagnosed at the age of 5 years, a black female presented with signs of increased intracranial pressure. Neuroimaging showed a large enhancing extra-axial occipital tumor mass. The resection specimen showed morphologic, cytogenetic, and immunophenotypic features consistent with relapse of the primary leukemia. Bone marrow examination was negative for malignancy. The long duration of complete remission followed by the formation of a mass in the central nervous system are highly unusual features of recurrent acute lymphoblastic leukemia. © 1996 Wiley-Liss, Inc.     

Differential diagnosis of acute central nervous system infections in children using modern microbiological methods

Aim:  Except bacterial meningitis, the agents causing acute central nervous system (CNS) infections in children are disclosed in only approximately half of the cases, and even less in encephalitis. We studied the potential of modern microbiological assays to improve this poor situation.
Methods:  In a prospective study during 3 years, all children attending hospital with suspected CNS infection were examined using a wide collection of microbiological tests using samples from the cerebrospinal fluid, serum, nasal swabs and stool.
Results:  Among 213 patients, 66 (31%) cases suggested CNS infection and specific aetiology was identified in 56 patients. Of these microbiologically confirmed cases, viral meningitis/encephalitis was diagnosed in 25 (45%), bacterial meningitis in 21 (38%) and neuroborreliosis in 9 (16%) cases while 1child had fungal infection. In meningitis patients, the causative agent was identified in 85% (35/41) cases and in encephalitis in 75% (12/16). The most common bacteria were Streptococcus agalactiae , Streptococcous pneumonie and Neisseria meningitidis, while the most frequently detected viruses were enteroviruses and varicella zoster virus.
Conclusion:  In 75% to 85% of paediatric CNS infections, specific microbiological diagnosis was obtained with modern laboratory techniques. The results pose a basis for prudent approach to these potentially serious diseases.     

儿童造血干细胞移植后中枢神经系统并发症的临床分析

目的：探讨儿童造血干细胞移植（HSCT）后中枢神经系统（CNS）并发症的发病情况、病因、临床特点、高危因素及预后,提高 CNS 并发症的诊断和治疗水平,改善患儿的生存质量。方法回顾性分析113例行HSCT治疗的患儿发生癫痫、高血压脑病、可逆性后部白质脑病综合征和移植相关的血栓性微血管病等HSCT后CNS并发症的诱因、发病特点及预后。结果113例行HSCT治疗患儿中共7例（6.2％）发生了CNS并发症,其中1例死亡。7例患儿中,6例为HLA不全相合,1例患儿为HLA全相合。7例患儿在预处理时均应用ATG。结论 VHLA配型不全相合可能是HSCT后发生CNS并发症的高危因素。早期发现、早期诊断并积极治疗CNS 并发症,可降低其病死率及后遗症的发生,有效改善患儿的生存质量。