急性T淋巴细胞白血病患儿中枢神经系统白血病的防治

急性T淋巴细胞白血病(T-ALL)占儿童急性淋巴细胞白血病的12%~15%。据文献报道,约有9.5%的T-ALL患儿于起病时已有中枢神经系统浸润,明显高于急性B淋巴细胞白血病B-ALL(约3%患儿初治即有中枢神经系统浸润)。中枢神经系统白血病(CNSL)的防治主要包括鞘内化疗、全身系统化疗、颅脑放疗三个方面。许多协作组,已经对包括CNSL在内的所有T-ALL患儿均省略了颅脑放疗。部分协作组,采用危险系数评估对部分存在高危因素的患儿进行颅脑放疗。但目前尚无明确数据显示,颅脑放疗可被鞘内化疗及全身化疗所代替。因此,评估放疗的风险、患儿的生存率和生存质量以及患儿、患儿家长的意见是我们选择治疗方案的一项重要参考。     

Detection of central nervous system leukemia in children with acute lymphoblastic leukemia by real-time polymerase chain reaction

Accurate detection of central nervous system (CNS) involvement in children with newly diagnosed acute lymphoblastic leukemia (ALL) could have profound prognostic and therapeutic implications. We examined various cerebrospinal fluid (CSF) preservation methods to yield adequate DNA stability for polymerase chain reaction (PCR) analysis and developed a quantitative real-time PCR assay to detect occult CNS leukemia. Sixty CSF specimens were maintained in several storage conditions for varying amounts of time, and we found that preserving CSF in 1:1 serum-free RPMI tissue culture medium offers the best stability of DNA for PCR analysis. Sixty CSF samples (30 at diagnosis and 30 at the end of induction therapy) from 30 children with ALL were tested for CNS leukemic involvement by real-time PCR using patient-specific antigen receptor gene rearrangement primers. Six of thirty patient diagnosis samples were PCR-positive at levels ranging from 0.5 to 66% leukemic blasts in the CSF. Four of these patients had no clinical or cytomorphological evidence of CNS leukemia involvement at that time. All 30 CSF samples drawn at the end of induction therapy were PCR-negative. The data indicate that real-time PCR analysis of CSF is an excellent tool to assess occult CNS leukemia involvement in patients with ALL and can possibly be used to refine CNS status classification.     

纤维连接蛋白在小儿急性淋巴细胞白血病中枢神经系统白血病脑脊液中的表达及意义

目的观察纤维连接蛋白(FN)在小儿急性淋巴细胞白血病中枢神经系统白血病(ALL-CNSL)脑脊液(CSF)中的基因表达水平,探讨其与髓外浸润及临床特征的关系。方法应用实时定量聚合酶链反应(RT-PCR)和间接免疫荧光试验检测100例ALL-CNSL患者的CSF(试验组)和骨髓(对照组)中的FN基因表达情况,并结合临床资料进行分析。结果经RT-PCR检测,FN在CSF中表达量明显高于骨髓中的表达,两组差异具有统计学意义(4.055±1.134 vs.0.053±0.048,P<0.05)。其中白细胞数≥5×106/L患儿的CSF中FN表达量比白细胞数<5×106/L患儿CSF中的表达明显增多,差异具有统计学意义(5.250±2.046 vs.3.023±1.018,P<0.05)。而CSF中FN的表达量与年龄、性别和ALL的类型无相关性;间接免疫荧光试验检测到CSF原始淋巴细胞FN分布在细胞内膜,其阳性表达率78.0%,骨髓原始淋巴细胞FN的表达为阴性;FN的RT-PCR检测与间接免疫荧光法检测两种方法之间具有显著相关性(P<0.05),但一致性较差(κ=0.27)。结论 FN在ALL-CNSL患者的CSF中呈现高表达,其与CSF中白细胞的负荷量存在相关性,FN的表达与ALL的中枢神经系统浸润有关。     

成人急性髓细胞白血病血小板输注阈值的探讨

目的　探讨急性髓细胞白血病诱导缓解化疗期间血小板输注的阈值。　方法　分析本院 3年来急性髓细胞白血病 (M3除外 )诱导缓解期间出血危险因素、预防性血小板输注及临床出血情况。　结果　 1 1 7例患者诱导缓解化疗1 78次 ,共输注机采血小板 1 61次。在患者血小板计数 <5× 1 0 9/L时输注血小板 ,临床主要出血症状增加。　结论　在患者状态稳定情况下 ,5× 1 0 9/L作为血小板输注的阈值是安全的 ,有高危出血因素时 (如患者发热 >3 8.5℃或有严重感染或化疗前高白细胞等 )血小板输注的阈值应为 1 5× 1 0 9/L或更高     

How does leukemia invade the central nervous system?

Prolongation of survival of children with acute leukemia by systemic therapy has brought a marded increase in incidence of CNS leukemia. In mice inoculated with leukemia cells, CNS i nfiltrates are rare unless life is prolonged by chemotherapy. Available evidence seems to indicate that leukemic cells in the CNS in most cases originate in bone marrow or lymph nodes.     

Cognitive consequences and central nervous system injury following treatment for childhood leukemia

OBJECTIVES: To determine the relationship between membrane damage and intellectual and academic abilities in children with acute lymphoblastic leukemia (ALL) and pilot test a math intervention for children with ALL who were affected. DATA SOURCES: Research studies and review articles. CONCLUSIONS: Despite the prophylactic central nervous system (CNS) treatment for long-term disease-free survival, many children with ALL subsequently experience declines in intellectual and academic skills. IMPLICATIONS FOR NURSING PRACTICE: Improving academic abilities in children who have received CNS treatment is of high priority and may have longlasting implications on quality of life.     

Primary isolated central nervous system acute lymphoblastic leukemia with BCR-ABL1 rearrangement: A case report

BACKGROUND BCR-ABL1 fusion gene is associated with a poor prognosis and a high incidence in central nervous system (CNS) leukemia. CNS invasion which detected at the initial diagnosis is commonly with bone marrow infiltration. It is uncommon for the leukemia cells to be located primarily in the CNS without bone marrow involvement.CASE SUMMARYWe here report the rare initial presentation of CNS-restricted BCR-ABL-positive acute lymphoblastic leukemia in a 30-year-old female patient who clinically manifested with leukemic meningitis, with no involvement in peripheral blood or bone marrow. Identification of abnormal phenotypes of blast cells, and BCR-ABL1 rearrangement in the cerebrospinal fluid alone established the diagnosis of primary CNS-isolated acute lymphocytic leukemia. The patient received a combination of intrathecal therapy and high-dose chemotherapy. But the benefits of the treatments were short-lived and she experienced recurrence.CONCLUSIONFlow cytometry in combination with molecular genetic analysis improved diagnostic accuracy. New approaches that may enhance the efficacy of the existing therapies and cure CNS leukemia are required.     

脑脊液流式细胞学检测技术在急性淋巴细胞白血病儿童并发中枢神经系统白血病中的诊断价值

本研究旨在探讨脑脊液流式细胞学检测(FCM)技术对儿童急性淋巴细胞白血病(ALL)并发中枢神经系统白血病(CNSL)的诊断价值。采用FCM技术、脑脊液常规细胞学(CC)涂片法、脑脊液常规及生化检测法对75例初诊ALL患儿首次脑脊液标本进行检测,并以9例病毒性脑炎患儿初次脑脊液检测结果作为对照。结果表明,75例初诊ALL患儿,中位发病年龄5(1-14)岁,男女比例1.3∶1。根据CCLG-ALL2008疾病危险度分组:标危组38例,中危组22例,高危组15例。首次脑脊液检查结果显示:FCM+CC+5例(6.7%),根据CCLG-2008中枢神经系统(CNS)状态分级:CNS-2 3例,CNS-3 2例;FCM+CC-8例(10.7%),其中CNS-1 7例,CNS-2 1例;FCM-CC-62例(82.7%),其中CNS-1 60例,CNS-2 1例,CNS-3 1例。本研究结果显示,FCM+CC+,FCM+CC-,FCM-CC-3组在CNS-1,-2,-3分布上无显著性差异(P>0.05),考虑可能与样本数量偏小有关。9例病毒性脑炎患儿初次脑脊液CC涂片中均未见肿瘤细胞。结论:脑脊液FCM技术敏感度高,在CNS-2,CNS-3诊断上与脑脊液CC检查具有较高的一致性,并可将CC诊断的CNS-2,-3阳性率提高20%,是脑脊液CC检测方法的重要补充,在儿童ALL并发CNSL的诊断中具有重要的应用价值。     

Late results of the treatment of acute granulocytic leukemia in adults

Eighteen patients with acute granulocytic leukemia (MI-1, M2-10, M3-1 and M4-6) underwent AdOAP treatment. Complete remissions were achieved in 10 out of the 18 patients (55.6%), 3 patients proved resistant, 5 had died prior to recovery of normal hemopoiesis. Three lethal cases are attributed to the results of the therapy complications. Median duration of complete remissions reached 17.5 months. Two patients have been in remission over 3 years. Reduced duration or intensity of the induction therapy failed to diminish frequency of lethal complications due to progressive cytopenia.     

Infection with involvement of the central nervous system

A detailed clinical characterization of 124 cases of sepsis with involvement of the central nervous system is presented and basic clinicopathogenic variants of its affection in sepsis, problems of diagnosis, etiotropic and pathogenic therapy are discussed.