儿童急性白血病合并中枢神经系统真菌感染5例并文献复习

目的通过5例儿童急性白血病合并中枢神经系统(CNS)真菌感染的临床总结及相关文献复习,提高对本病的认识。方法回顾性分析北京儿童医院收治的5例急性白血病合并CNS真菌感染患儿的临床特点及诊治经过并文献复习。结果(1)4例急性淋巴细胞白血病(ALL)患儿均接受强化疗,1例为急性非淋巴细胞白血病(AML-M2)半相合造血干细胞移植术后,发生CNS真菌感染前均有肺部真菌感染病史,其中2例合并肝、脾、肾真菌感染,1例合并下肢皮肤真菌感染(2)实验室检查3例有病原学依据,1例烟曲霉菌,2例念珠菌,2例无病原学依据。(3)影像学5例患儿均作头颅CTMRI,表现脓肿、梗塞、颅内出血。(4)治疗及预后1例患儿伊曲康唑+脂溶性两性霉素B治疗,2例患儿伏立康唑治疗,2例患儿伏立康唑和两性霉素B联合抗真菌治疗,抗真菌治疗均有效,4例存活,1例死于严重移植物抗宿主病(GVHD)。结论儿童急性白血病合并CNS真菌感染为侵袭性真菌感染的表现形式之一,多半有其他脏器感染,临床表现不典型,可有脑膜炎或脑脓肿症状和体征,诊断困难,CT、MRI影像学检查重要,伏立康唑、两性霉素B抗真菌治疗有效。     

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。     

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.     

婴儿中枢神经系统白色念珠菌病5例并文献复习

目的 总结婴儿中枢神经系统白色念珠菌病的临床特点和诊断治疗经验.方法 收集首都医科大学附属北京儿童医院2009至2011年收治的婴儿中枢神经系统白色念珠菌病连续病例(5例)的临床资料,总结其临床表现、实验室检查、影像学特征、治疗和随访情况,并文献复习.结果 男4例,女1例,年龄3～5月龄(平均4月龄),均无明显免疫缺陷...     

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     

儿童急性白血病化疗合并真菌感染探讨

目的探讨儿童急性白血病化疗合并真菌感染的因素及其感染病原学特点。方法白血病并发真菌感染按EORTC诊断标准。分析真菌感染率、感染部位、感染相关危险因素、病原菌特点。结果共17例白血病患儿21次并发真菌感染,急性淋巴细胞性白血病(ALL)13例,急性非淋巴细胞性白血病(AML)4例;合并真菌感染率为27.6%。感染部位主要位于上呼吸道(61.9%)。病原菌培养均示浅表真菌,如白色念珠菌、酵母样菌。化疗阶段是白血病患儿合并真菌感染的重要因素(P<0.05),免疫功能和中性粒细胞绝对值在临床上亦是主要的影响因素。结论白血病患儿合并真菌感染与疾病本身、机体免疫功能低下、中性粒细胞绝对值和化疗阶段相关。因此,免疫支持、早期诊断、及时治疗颇为重要。     

儿童中枢神经系统白血病的诊治进展

鉴于急性白血病联合化疗时血脑屏障的作用,白血病细胞逃避化疗药物的杀伤最终导致中枢神经系统白血病(central nervous system leukemia,CNSL)发生.目前诊断CNSL的标准主要是依赖于脑脊液(carebrospinal fluid,CSF)检查、临床表现和影像学检查,早期诊断方法有CSF细胞形态学检查、CT和MRI等影像学检查、CSF微小残留病的检测、检测CSF硫化氢含量、分子生物学指标的检测.CNSL的预防主要包括直接鞘内注射药物、可透过血脑屏障的全身化疗药物和放射疗法.CNSL的治疗主要包括化疗放疗、异基因造血干细胞的移植和靶向治疗.     

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。     

Isolated central nervous system recurrence of acute promyelocytic leukemia in children

The incidence of isolated central nervous system (iCNS) relapse in pediatric acute promyelocytic leukemia (APL) is debated. We analyzed the literature, focusing on clinical trials reported since the advent of ATRA use. Only 2/218 (0.92%) good risk patients (diagnostic WBC <10,000/microl) had truly iCNS relapse. This incidence does not support the use of intrathecal CNS prophylaxis for all children with APL. We also identified multiple deficiencies in these reports. Additional reporting of these events could provide insight into the true incidence and pathogenesis of iCNS relapse and might allow for identification of risk factors associated with such extramedullary relapse.     

Neuropsychological status of children with acute lymphoblastic leukemia treated for central nervous system relapse

Approximately 10% of children treated with contemporary therapy for acute lymphoblastic leukemia (ALL) will experience an isolated relapse in the central nervous system (CNS). From 5 to 25% of this group will become long-term survivors, but only after additional, more aggressive therapy. A review of the limited number of studies of children surviving treatment for CNS relapse disclosed a strikingly higher incidence of intellectual impairment than was found in similarly treated patients who remain in complete remission. Specific risk factors for this complication included the number of courses of cranial irradiation, a young age at treatment, increasing time since treatment, neuropathological changes apparent on abnormal computed tomography scans of the brain, and seizures. The methodological problems inherent in neurobehavioral research in childhood acute lymphoblastic leukemia are critically reviewed and suggestions for future studies are offered. Children with a CNS relapse should be serially evaluated for psychoeducational performance to facilitate early intervention in cases of learning difficulties.     

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。     

Radiotherapy of the central nervous system in acute leukemia

The central nervous system (CNS) is a site of occult and overt involvement with acute lymphoblastic leukemia (ALL) in children. Prophylactic treatment of the cranial and spinal meninges can significantly reduce the incidence of CNS relapse. This review addresses the issues associated with the role of radiation therapy in the treatment of the CNS in ALL.     

Late sequelae of central nervous system prophylaxis in children with acute lymphoblastic leukemia: high doses of intravenous methotrexate versus radiotherapy of the central nervous system--review of literature

Acute lymphoblastic leukemia is the most common malignancy in children. All current therapy regimens used in the treatment of childhood acute lymphoblastic leukemia include prophylaxis of the central nervous system. Initially it was thought that the best way of central nervous system prophylaxis is radiotherapy. But despite its effectiveness this method, may cause late sequelae and complications. In the programme currently used in Poland to treat acute lymphoblastic leukemia, prophylactic radiotherapy has been reduced by 50% (12 Gy) and is used only in patients stratified into the high risk group and in patients diagnosed as T-cell ALL (T-ALL). Complementary to radiotherapy, intrathecal methotrexate is given alone or in combination with cytarabine and hydrocortisone is given, as well as systemic chemotherapy with intravenous methotrexate is administered in high or medium doses (depending on risk groups and leukemia immunophenotype). Recent studies have shown that high dose irradiation of the central nervous system impairs cognitive development causing memory loss, visuomotor coordination impairment, attention disorders and reduction in the intelligence quotient. It has been proved that the degree of cognitive impairment depends on the radiation dose directed to the medial temporal lobe structures, particularly in the hippocampus and the surrounding cortex. Also, methotrexate used intravenously in high doses, interferes with the metabolism of folic acid which is necessary for normal development and the optimal functioning of neurons in the central nervous system. It has been proved that patients who have been treated with high doses of methotrexate are characterized by reduced memory skills and a lower intelligence quotient. The literature data concerning long term neuroanatomical abnormalities and neuropsychological deficits are ambiguous, and there is still no data concerning current methods of central nervous system prophylaxis with low doses of irradiation in combination with high doses of intravenous methotrexate.