Efficacy of therapy of different variants of anaplastic large T-cell lymphomas

AIM: To compare efficacy of NHL-BFM-90 and CHOP-like courses in the treatment of anaplastic large cell lymphoma (ALCL). MATERIAL AND METHODS: Twenty-two patients with ALCL participated in the study. The diagnosis was made basing on the findings of clinical, device, morphological, immunohistochemical and molecular-genetic examinations with application of a panel of monoclonal antibodies to CD30, ALK, CD3, CD4, CDS, CD7, CD34, CD15, CD68, CD20, CD45RO, CD45RA, Ki-67. 14 cases of 22 were negative by kinase of anaplastic lymphocytes (ALK-) and 8 were positive (ALK+). Mean age of ALK-ALCL patients was 39.6 +/- 4.1 years, of ALK+ALCL patients - 23.4 +/- 2.6 years. 14 patients were treated by the protocol NHL-BFM-90, 8 were initially treated with other schemes (CHOP, MACOP-B, BEACOPP and others). All 14 patients treated according to NHL-BFM-90 had ALCL stages III-IV with B-symptoms. 12 patients who completed treatment by the above protocol achieved complete remission after the forth course, 2 patients failed the treatment. Of 8 ALCL patients treated initially according to other schemes, a complete remission was achieved in 4 patients (2 had stage II). One of 4 patients with remission had recurrence. Four patients who had failed to achieve complete remission died of the disease progression. CONCLUSION: ALCL occurs more frequently in young and middle-aged patients. The disease has an aggressive course with rapid generalization. For such processes it is more preferable to use a modified protocol NHL-BFM-90.     

Use of Mabtera (rituximab) in treating patients with refractory courses of B-cell lymphoma,along with high-dose chemotherapy and autologous transplantation of hematopoietic stem cells

AIM: To study efficacy of rituximab in patients with resistant B-cell lymphoma on high-dose chemotherapy. MATERIAL AND METHODS: From September 2000 to April 2002 we studied efficacy and tolerance of rituximab at different stages of high-dose chemotherapy. The treatment was given to 10 patients with histologically verified CD20+ non-Hodgkin's lymphoma: diffuse large-cell (n = 4), Berkitt's (n = 2), follicular (n = 3), mantle-cell (n = 1). Five patients with diffuse large-cell lymphoma and Berkitt's lymphoma had a primary resistant course of the disease, one patient with diffuse large-cell lymphoma had a refractory recurrence. Follicular and mantle-cell lymphomas were characterized by a resistant course and large tumor masses. The patients received 1-2 courses of induction chemotherapy with dexa-BEAM with collection of peripheral stem cells followed by high-dose chemotherapy (BEAM-9, CBV + mitoxantron-1) with transplantation of autologous stem blood cells. Rituximab infusion (375 mg/m2) was conducted before the collection of the stem cells, prior to high-dose chemotherapy and in posttransplantation period after recovery of hemopoiesis. RESULTS: 4 patients achieved complete remission, 3-partial remission, 2 had progression and 1-stabilization. In mean follow-up 11 (2-20) months 7 of 10 patients were alive, overall survival being 15 +/- 2.4 months (95% confidence interval 10-19.7), median was not reached. 5 patients are in complete remission: 2 of them without further treatment, 3-after progression and repeat therapy including rituximab and interferon-alpha or rotuximab and CHOP chemotherapy. CONCLUSION: The addition of rituximab can improve the results of high-dose chemotherapy of patients with non-Hodgkin's lymphoma resistant to standard doses of cytostatics. Repeat use of this drug can be effective in some patients with progression after high-dose chemotherapy with rituximab.     

Treatment of adult Berkitt-like lymphoma

AIM: To compare programs of chemotherapy used in adult Berkitt-like lymphoma (ABLL); to assess efficacy and toxicity of the protocol AblL-M-04. MATERIAL AND METHODS: 31 ABLL patients (23 males, 8 females, mean age 27 years) participated in the study performed in Hematological Research Center in 1995-2004. ABLL stage I, II, III and IV was diagnosed in 3, 5, 8 and 15 patients, respectively. 10 patients had diffuse large B-cell lymphoma. 9 patients received 2 to 6 courses of CHOP, 1 patient--6 courses of Pro-Mace-Cytabom, 11 patients with newly diagnosed ABLL and 5 pretreated with CHOP--NHL-BFM-90. The modified protocol ABLL-M-04 of intensive short-term therapy included 10 patients, 2 of them pretreated. RESULTS: Of 10 patients given CHOP or CHOP-like courses 9 were resistant to therapy, 2 died of rapid progression, 7 were converted to the program therapy. 5 patients on the protocol NHL-BFM-90 died after short-term improvement. None of them achieved remission. Of 10 patients with newly diagnosed ABLL treated according to NHL-BFM-90 protocol, remission was achieved in 4 patients, follow-up median--34 months (2-56). Six patients died: 4 of progression, 2 of chemotherapy complications. BLL-M-04 therapy was made in 9 patients: 7 patients persisted on the first remission, 2 patients died of chemotherapy complications. Overall duration of the treatment was 3-3.5 months. CONCLUSION: The protocol ABLL-M-04 seems to be more effective than a classic NHL-BFM-90, but this must be supported by more cases. CHOP therapy cannot be recommended for patients with ABLL because of poor efficacy (all the CHOP patients died).     

HLH-2004方案治疗继发性噬血细胞性淋巴组织细胞增多症10例临床分析

本研究旨在探讨HLH-2004方案在继发性噬血细胞性淋巴组织细胞增多症（secondary hemophagocytic lymphohistiocytosis,sHLH）患者中的临床疗效。对我科10例行HLH-2004方案化疗的sHLH患者进行回顾性分析。结果表明,化疗中7例患者有临床反应,3例患者无反应。5例sHLH患者未完成8周初始治疗,其中4例死亡,1例因严重药物副反应改用CHOP方案,治疗4个疗程后获疾病缓解。5例患者完成初始治疗,3例获疾病缓解,2例疾病控制不佳。3例缓解患者中,1例死于疾病复发,另2例维持缓解;2例控制不佳的患者中,1例死亡,另1例出院后获疾病缓解。结论：感染相关的HLH（IAHS）患者予HLH-2004方案化疗联合有效抗感染治疗后缓解率高,而EB病毒相关的HLH（EBV-HLH）或淋巴瘤相关的HLH（LAHS）患者缓解率低、缓解后易复发。     

减低剂量利妥昔单抗联合CHOP方案治疗老年弥漫性大B细胞淋巴瘤的疗效观察

目的 观察减低剂量利妥昔单抗+环磷酰胺+多柔比星+长春新碱+泼尼松(R-CHOP)方案与减低剂量环磷酰胺+多柔比星+长春新碱+泼尼松(CHOP)方案治疗老年弥漫性大B细胞淋巴瘤(DLBCL)的临床疗效及不良反应.方法 选取2007年1月至2013年6月,四川省医学科学院·四川省人民医院血液科收治的老年DLBCL患者66例为研究对象.全部患者均经组织病理学及免疫组织化学检测,且均符合《世界卫生组织造血及淋巴组织肿瘤分类(2008年版)》中关于DLBCL的诊断标准,明确诊断为DLBCL.本研究纳入标准:①符合DLBCL的诊断标准;②年龄＞60岁;③Ann Arbor临床分期为Ⅱ～Ⅳ期.排除标准:①患者入院前已接受其他药物或手术治疗;②患者在本次入院前已合并严重的肝、肾疾病,以及恶性肿瘤等严重系统性疾病;③患者对本次治疗应用的药物有变态反应史.采用简单随机法将66例老年DLBCL患者分为2组,分别为减低剂量R-CHOP方案化疗组(n=27)与减低剂量CHOP方案化疗组(n=39).66例初治老年DLBCL患者66例应用减低剂量CHOP或R-CHOP方案进行化疗,每例患者治疗至少3个周期评估疗效,能够耐受减低剂量R-CHOP或CHOP方案的老年DLBCL患者,行化疗4～6个周期,即1个疗程.对老年DLBCL患者进行近期治疗反应的评估,并随访观察总生存(OS)率,无进展生存(PFS)率及无事件生存(EFS)率.并观察老年DLBCL患者接受减低剂量CHOP或R-CHOP方案进行化疗后的不良反应.本研究遵循的程序符合四川省医学科学院·四川省人民医院人体试验委员会所制定的伦理学标准,得到该委员会批准,征得受试对象的知情同意,并与之签署临床研究知情同意书.两组患者年龄、性别构成比等一般临床资料比较,差异均无统计学意义(P＞0.05).结果 ①减低剂量R-CHOP方案化疗组27例老年DLBCL患者中,1例患者因合并症而终止化疗,其余26例患者化疗后的完全缓解(CR)率为69.2％(18/26),部分缓解(PR)率为19.2％(5/26),总缓解(OR)率为88.5％(23/26).减低剂量CHOP方案化疗组39例老年DLBCL患者中,1例患者化疗1个周期后猝死,1例化疗后因合并症而终止化疗,其余37例患者化疗后的CR率为43.2％(16/37),PR率为21.6％(8/37),OR率为64.7％(24/37).减低剂量R-CHOP方案化疗组CR率、OR率均高于减低剂量CHOP化疗方案组,且差异有统计学意义(x2=4.15、4.49,P＜0.05);但两组患者PR率相比,差异却无统计学意义(x2=0.05;P＞0.05).②本组中位随访时间为42个月,随访率为95.5％.减低剂量R-CHOP方案化疗组可评估的26例老年DLBCL患者的3年PFS率为53.8％(14/26),3年EFS率为57.7％(15/26),3年OS率为80.8％(21/26).减低剂量CHOP方案化疗组可评估的37例老年DLBCL患者的3年PFS率为51.4％(19/37),3年EFS率为51.4％(19/37),3年OS率为56.8％(21/37).减低剂量R-CHOP方案化疗组老年DLBCL患者3年OS率高于减低剂量CHOP方案化疗组患者,且差异有统计学意义(x2=3.96,P＜0.05);两组患者3年PFS率、3年EFS率相比,差异却均无统计学意义(x2=0.04、0.25,P＞0.05).③两组老年DLBCL患者接受减低剂量CHOP或R-CHOP方案进行化疗后常见化疗相关不良反应为粒细胞计数减低、血小板计数减低、贫血、合并感染、胃肠道反应、肝功能异常、心脏不良反应.减低剂量R-CHOP方案组与减低剂量CHOP方案化疗组不良反应率分别为38.5％(10/26)与40.5％(15/37),两者相比,差异无统计学意义(P＞0.05).结论与减低剂量CHOP化疗方案相比,减低剂量R-CHOP化疗方案可以提高老年DLBCL患者的OR率、CR率、并提高OS率,可作为初治老年DLBCL患者的化疗方案之一.     

Prognostic evaluation of early detection of relapse in adult acute leukemia

A criterion was established in a previous study to detect the relapse of adult acute leukemia in its early stage, i.e., when lymphocytes in peripheral blood increased over 45% of the leukocytes during the maintenance therapy, bone marrow puncture was immediately performed to examine the leukemic cells. By this criterion the relapse was detected earlier than by other criteria. To study the effect of early detection of the relapse in adult acute leukemia on the results, the second remission rate and the survival time were compared between the following groups. Group I consisted of 11 patients, whose relapse was determined by our criterion. Group II consisted of 8 patients whose relapse was determined by the appearance of leukemic cells in peripheral blood. The second remission was accomplished in 7 of 11 patients in Group I (64%) and in 1 of 8 in Group II (13%) (p less than 0.05). The mean (+/- S.D.) duration of complete remission in Group I was 9.7 +/- 7.8 months and not significantly longer than the value in Group II (5.3 +/- 3.9 months). The interval from relapse to death was 10.3 +/- 5.9 and 6.1 +/- 3.8 months in Groups I and II, respectively. The interval from relapse to death of 7 patients who accomplished the second remission in Group I was 12.5 +/- 5.5 months. This interval was significantly longer than in Group II (p less than 0.02). The mean survival time was 22.2 +/- 9.6 months in Group I and 13 +/- 2.5 months in Group II. The mean survival time of 7 patients accomplished the second remission in Group I was 26. 7 +/- 9.4 months which was significantly longer than in Group II (p less than 0.01). The results showed that our criterion to detect relapse in the early stage was effective for prolongation of the survival time in adult acute leukemia.     

负载自体肿瘤抗原的DC-CIK细胞联合自体造血干细胞移植治疗难治性淋巴瘤的临床研究

目地观察负载自体肿瘤抗原的DC-CIK细胞联合自体造血干细胞移植治疗难治性淋巴瘤的疗效。方法选取难治性淋巴瘤35例,采用MAC预处理方案,用自体淋巴瘤抗原致敏DC-CIK细胞,于移植预处理后5—10d,将DC-CIK细胞回输给患者。结果35例难治性淋巴瘤中,29例完全缓解(82.86%),4例部分缓解(14.43%),移植过程中死亡2例(5.71%)(均死于严重混合性感染)。所有完全缓解和部分缓解病例均随访3—49个月:4名部分缓解患者分别于移植后3、6、10、13个月后病情进展死亡;完全缓解患者中有3人于移植后11、17、20个月再次复发死亡;现存活26例。结论负载自体肿瘤抗原的DC-CIK细胞联合自体造血干细胞移植治疗难治性淋巴瘤高于单纯自体外周血造血干细胞的疗效,且无明显毒副作用。     

PDD方案治疗初治多发性骨髓瘤的临床研究

目的：观察硼替佐米联合脂质体多柔比星和地塞米松（PDD）方案治疗初治多发性骨髓瘤（MM）的疗效。方法回顾性分析26例初治MM,采用PDD方案：硼替佐米1.3 mg/m2,第1、4、8、11天,脂质体多柔比星40 mg,第1天,地塞米松20 mg,第1-2,4-5,8-9,11-12天,每4周1个疗程,共2-6个疗程,观察疗效和不良反应。结果平均接受（4.1±1.1）（2-6）个疗程,有效率为25例（96%）,2个疗程有效率23例（88%）。26例2个疗程达严格意义完全缓解（sCR）4例（15%）,完全缓解（CR）8例（31%）,非常好的部分缓解（VGPR）8例（31%）,部分缓解（PR）3例（12%）,轻微反应（MR）2例（8%）,疾病稳定（SD）1例（4%）。骨髓瘤细胞、血清单克隆M蛋白均较治疗前下降。最常见不良反应为血液学改变,血小板减少、中性粒细胞减少、贫血;其次为胃肠道反应,便秘。另外,周围神经病变（PN）较为多见。结论PDD方案治疗初治MM具有良好的临床疗效和安全性。     

141例儿童急性髓系白血病的疗效及预后相关因素分析

目的评价初治儿童急性髓系白血病(AML)的疗效及探讨除急性早幼粒细胞白血病(APL)外的 AML 的预后相关因素。方法 141例18岁以下 AML 患者分成 APL 组(A 组,51例)和除APL 外的 AML 组(B 组,90例)进行回顾性研究分析。采用 Kaplan-Meier 曲线评估患者的无事件生存(EFS)率、无病生存(DFS)率和总生存(OS)率,Cox 回归模型评估预后因素。结果 B 组1个疗程完全缓解(CR)率为54.4%(49例),总缓解率为76.7%。5年累积 EFS 率、DFS 率和 OS 率分别为(28.4±9.0)%、(28.39±8.96)%和(35.5±6.3)%;A 组5年累积 EFS 率、DFS 率和 OS 率分别为(81.5±5.7)%、(94.3±4.0)%和(81.4±5.7)%:全部141例 AML 患儿5年累积 DFS 率和5年累积OS 率分别为(56.9±6.3)%和(53.3±4.8)%。B 组病例经多因素分析表明,初诊时骨髓白血病细胞比例较高和≥2个疗程达 CR 以及巩固治疗6个疗程以下是影响患者预后的危险因素(P 值均<0.05)。结论儿童 APL 预后良好。其他儿童 AML 中,初诊时骨髓原始细胞比例低和1个疗程达CR 以及巩固治疗6个疗程以上者预后较优;儿童 M_(2h)/t(8;21)与除 APL 以外的其他亚型相比没有显示预后良好的趋势;降低复发是改善儿童 AML 预后的关键。     

血红蛋白浓度与食管癌放射治疗患者局部控制率及远期生存率的相关性

目的 探讨血红蛋白(Hb)浓度与食管癌放射治疗患者局部控制率及远期生存率的关系.方法 选取2016年1月至2017年1月在我院治疗的食管癌患者88例,均接受放疗治疗,观察随访患者治疗疗效,检测放疗前后Hb水平.结果 经放疗后,完全缓解(CR)患者8例,部分缓解(PR)患者30例,稳定(SD)患者29例,疾病进展(PD)...     

预激诱导治疗急性髓细胞性白血病临床分析

目的:探讨预激诱导治疗对急性髓细胞白血病(AML)的临床疗效及毒副作用。方法:将AML43例分为2组。常规化疗组17例,均为用经典方案诱导化疗2疗程以上未获完全缓解或首次完全缓解6个月内复发者;男7例,女10例,平均年龄43(17~65)岁。预激方案治疗组26例,其中14例为用经典方案诱导化疗2疗程以上未获完全缓解或首次完全缓解6个月内复发者,6例为MDS转化型,1例为继发性AML,5例为低增生性AML;男8例,女18例,平均年龄50.5(19~72)岁,60岁以上者有8例。主要方案:常规化疗方案(AA方案):阿克拉霉素(Acla)20 mg/d第1~5天,阿糖胞苷(Arac-C)150~200 mg/d第1~7天;预激方案(CAG方案):粒细胞集落刺激因子(G-CSF)150μg/d(与化疗同时进行,共10~14 d),Arac-C 30 mg/d,q12 h(共10~14 d),Acla 20 mg/d(第1、3、5、7、9天),WBC>30×109/L时停用G-CSF,治疗中出现骨髓Ⅳ度抑制时停止治疗。结果:常规化疗组,完全缓解(CR)4例,部分缓解(PR)3例,无效(NR)10例,有效率41.1%。预激方案治疗组,第1疗程后获CR 16例,PR 4例,NR 6例;PR中2例进行了第2疗程的预激治疗达CR,2例放弃治疗,有效率76.9%;8例老年白血病患者中CR 5例、PR 1例、NR 2例;5例低增生性白血病患者均获CR;CR后给予常规剂量强化治疗,除1例死于严重感染外其余均处于缓解状态。两组治疗有效率比较,预激方案治疗组明显高于常规化疗组。预激方案治疗组中有1例出现Ⅳ度骨髓抑制达1月,其余患者均能很好耐受。结论:G-CSF预激诱导治疗方案对难治及复发性AML患者具有良好的效果,且对老年AML及低增生AML同样具有较高的缓解率,是一种安全、有效的治疗方案。     

Effects of cyclophosphamide on the length of remission in patients suffering from glomerulonephritis with nephrotic syndrome

The authors performed retrospective evaluation of recurrence rate and the length of nephrotic syndrome (NS) remission in 21 patients with chronic glomerulonephritis (GN), who suffered from a first NS attack, cured by corticosteroid (CS) monotherapy; 16 patients had mesangioproliferative GN, 1--mesangiocapillary GN, 1--membranous GN, 3 patients did not undergo biopsy. After obtaining steroid remission of NS, 11 patients (Group I) continued receiving CS in maintaining doses during the next 6 7 +/- 1.0 months; the other 10 patients (Group II) received maintaining doses of CS during the next 5.5 +/- 0.5 months plus cyclophosphamide (CFA) daily or as pulse therapy during 19.7 +/- 3.5 months. There was almost a double increase of mean remission length in Group II vs. Group I (47.6 +/- 9.8 and 25.3 +/- 6.4 months, respectively). There was only one case of a NS relapse in Group II vs. 7 in Group I, which means that the risk of a NS relapse was 7.7 times higher in patients receiving only CS vs. patients on combination of CS and CFA, p = 0. 004. The results of this small retrospective study suggest that administration of CFA as supporting therapy after obtaining steroid remission of NS allows its substantial prolongation in patients with chronic GN.     

(18)F-fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Findings in Mucosa-associated Lymphoid Tissue Lymphoma of the Larynx: a Case Report and Literature Review

Laryngeal mucosa-associated lymphoid tissue (MALT) lymphoma is rare, with only 25 cases reported in the literature. This report presents a case of laryngeal MALT lymphoma in a 35-year-old female with a 6-year history of progressively worsening hoarseness. MALT lymphoma was diagnosed based on biopsy and immunohistochemical analysis. The patient received two cycles of cyclophosphamide + epirubicin + vincristine + prednisone (CHOP) chemo therapy, which was ineffective. (18)F-fluoro deoxy glucose ((18)F-FDG) positron emission tomography/computed tomography (PET/CT) showed (18)F-FDG accumulation in the larynx only and identified stage IE lymphoma. CHOP chemotherapy was terminated and the patient was treated with radiotherapy. After 3 months (total radiation dose 27 Gy), (18)F-FDG PET/CT scan showed that the laryngeal lesion was in complete remission. A review of the literature on the MEDLINE(?)/PubMed(?) databases regarding laryngeal MALT lymphoma and the use of PET/CT found that radiotherapy is the firstline treatment for stage I and II MALT lymphoma.     

Prevention of neuroleukemia by intrathecal administration of cytosar and methotrexate in acute lymphoblastic leukemia in adults

AIM: To find out whether efficacy of neuroleukemia (NL) prevention by intrathecal administration of cytosar and methotrexate in remission induction phase in adult patients with acute lymphoblastic leukemia (ALL) depends on the risk factors. MATERIALS AND METHODS: The study covered 68 ALL patients. The diagnosis was made by cytological, histological and cytochemical tests of the peripheral blood and bone marrow. Immunophenotyping was performed in 48 patients. The treatment followed the German protocol 04.89 in modification of the Hematological Research Center of the Russian Academy of Medical Sciences. Prevention of NL consisted in intrathecal administration of cytosar (30 mg), methotrexate (15 mg) and dexamethasone (4 mg) once a week for 6 weeks beginning on induction day 1, further in consolidation, reinduction and once in 3 months in maintenance. Radiation of the brain was not conducted. Treatment of leuroleukemia consisted of intrathecal administration of the above drugs twice a week up to normalization of the liquor with subsequent their administration 5 times and craniospinal radiation in a dose 36 Gy. Further intrathecal administrations were made according to the protocol. RESULTS: Correlation was not found between age of the patients and frequency of neuroleukemia onset, between neuroleukemia incidence and peripheral blood leukocytosis at diagnosis. Results of NL prevention with cytosar and methotrexate given intrathecally in induction of remission (14.6% of neurorecurrences) are comparable with the results of NL prevention by radiation of the brain with intrathecal administration of methotrexate obtained in the German cooperative trial. CONCLUSION: NL prevention in ALL adult patients by intrathecal cytosar and methotrexate in remission induction is effective.     

儿童急性淋巴细胞白血病治疗早期白血病细胞及微量残留病监测的预后价值

目的评价诱导治疗第19天及血液学完全缓解(CR)时骨髓存在形态学可辨认的原始、幼稚淋巴细胞数及微量残留病监测在儿童急性淋巴细胞白血病(ALL)治疗中的预后价值。方法以1998年1月1日至2003年5月31日进入ALLXH99方案的193例新诊治ALL患儿为研究对象。联合化疗第19天及诱导缓解治疗结束时行骨髓形态学检查以及血液学CR时用四色多参数流式细胞仪监测微量残留病。结果①诱导治疗第19天骨髓原始、幼稚淋巴细胞≥0.050与<0.050的患儿5年无事件生存(EFS)率分别为(42.59±14.28)%和(74.24±6.67)%,差异有统计学意义(P<0.01);②诱导缓解治疗结束达血液学CR时骨髓存在形态学可识别的原始、幼稚淋巴细胞与此时无形态学可辨认的原始、幼稚淋巴细胞患儿5年EFS率分别为(63.47±9.23)%和(76.41±6.09)%,差异有统计学意义(P<0.05);③诱导缓解治疗结束血液学CR时微量残留白血病细胞≥10-4与<10-4的患儿骨髓原始、幼稚淋巴细胞数比例的差异无统计学意义(P>0.05);诱导缓解治疗结束血液学CR时微量残留白血病细胞≥10-4与<10-4的患儿22个月EFS率分别为(23.81±20.26)%和(94.44±5.40)%,差异有统计学意义(P=0.001)。结论诱导治疗第19天骨髓原始、幼稚细胞数≥0.050在儿童ALL治疗中具有独立的预后价值;诱导治疗结束血液学缓解时     

ALL-XH-99方案治疗儿童急性淋巴细胞白血病158例疗效分析

目的　对 15 8例儿童急性淋巴细胞白血病 (ALL)患儿治疗结果进行分析 ,探讨如何进一步提高儿童ALL无事件生存 (EFS)率。方法　 15 8例ALL患儿均应用ALL XH 99方案治疗。采用Kap lan Meier方法评估患儿的EFS ,组间患儿EFS差异用Log rank检验。全部数据均通过SPSS软件处理。结果　本组 15 8例完全缓解 (CR)率为 96 .8% ,2年、3年、4年、5年EFS率分别为 (85 .9± 3.1) %、(83.0± 3.6 ) %、(80 .9± 4 .1) %、(72 .4± 7.8) % ;2年、3年、4年、5年无病生存 (DFS)率分别为 (87.5±3 2 ) %、(85 .9± 3.5 ) %、(83.7± 4 .1) %、(75 .3± 7.8) %。低危、中危、高危组 5年EFS率分别为 (88.9± 5 .5 ) %、(78.5± 8.0 ) %、(5 3.4± 10 .9) %。 15例 (10 .0 % )复发 ,复发的中位时间为 12个月 (3～ 4 9个月 ) ;初次单独骨髓复发 13例 (8.7% ) ,中枢神经系统复发 2例 (1.3% ) ,无睾丸白血病复发。治疗相关死亡 7例 (4.7% )。结论　采用ALL XH 99方案按早期连续适度化疗的原则治疗儿童ALL ,降低了化疗相关死亡 ,总体上提高了ALL患儿的 5年EFS率。     

Respiratory muscle training in restrictive thoracic disease: a randomized controlled trial

OBJECTIVE: To investigate the effects of respiratory muscle training (RMT) in patients with restrictive thoracic disorders and intermittent noninvasive positive-pressure ventilation (NPPV). DESIGN: Prospective randomized controlled trial. SETTING: Home-based RMT, with assessment in a primary care pulmonary center. PARTICIPANTS: Thirty patients with restrictive thoracic disorders; 28 patients completed the trial. INTERVENTION: Three months of RMT by isocapnic hyperpnea or sham training. MAIN OUTCOME MEASURES: Respiratory muscle strength and endurance, lung function, exercise performance, and health-related quality of life (HRQOL). RESULTS: After RMT, maximal inspiratory mouth pressure was increased (27.6%+/-36.5%, P=.013). In patients who could perform cycle ergometer testing (n=17), peak oxygen consumption (2.24+/-3.39mLxkg(-1).min(-1) vs -1.71+/-2.54mLxkg(-1).min(-1), P=.014) and maximal work rate (9.4+/-14.8W vs -5.1+/-10.8W, P=.043) increased relative to a control group. Similar differences occurred regarding changes of HRQOL (physical performance, 3.3+/-11.4 score vs -6.6+/-9.0 score; P=.012) and time of ventilator use (-0.6+/-1.2h/d vs 0.4+/-0.5h/d, P=.010). Lung volumes, 12-second maximum voluntary ventilation, 6-minute walking distance, and blood gases were unchanged. CONCLUSIONS: In patients with restrictive thoracic disorders and NPPV, RMT improved inspiratory muscle strength. Exercise performance and HRQOL were improved when the 2 groups were compared. RMT was practicable and safe despite severe respiratory impairment. Further evaluation, including different training intensities and modalities, seems warranted.     

A phase 1/2 study of autologous neuroblastoma tumor cells genetically modified to secrete IL-2 in patients with high-risk neuroblastoma

Autologous neuroblastoma (NB) tumor cells modified to secrete interleukin (IL)-2 (auto-IL-2) can be safely given to patients with advanced neuroblastoma and generate antitumor immune responses. As the benefits of tumor immunization may be greater in patients with minimal residual disease and thus rely on surrogate markers such as immune responses to measure effect, we studied the frequency of immune changes associated with vaccination. Thirteen patients (8 in first remission and 5 after treatment for recurrent NB) received 5 to 8 subcutaneous injections of auto-IL-2 at 0.3 x 10 cells/kg. The vaccine was well tolerated. Injection site biopsies revealed increased cellularity caused by infiltration of CD4 and CD8 lymphocytes, eosinophils, and dendritic cells. Enzyme-linked immunosorbent spot assays for interferon-gamma and IL-5 demonstrated that vaccination produced a rise in circulating CD4 and CD8 T cells responsive to stimulation by autologous tumor cells. Median event-free survival was 22 months for patients in first remission and 3 months for all others. Four patients treated in first remission remain alive and 3 without disease recurrence.     

AML-XH-99方案治疗儿童急性髓系白血病82例疗效分析

目的　评估AML XH 99方案治疗儿童急性髓系白血病 (AML)的疗效 ,探讨如何进一步提高儿童AML无事件生存 (EFS)率。方法　 82例AML患儿均应用AML XH 99方案治疗。采用Kaplan Meier方法评估患儿的EFS ,全部数据通过SPSS软件处理。 结果　 1个疗程达完全缓解 (CR)者 5 8例 (70 .7% ) ,总CR率为 84 .1%。 5年EFS率和无病生存 (DFS)率分别为 (4 6 .1± 9.1) %和 (5 4 .3± 10 .3) %。 5 6例接受大剂量阿糖胞苷 (HD Ara C)强化治疗 ,其 5年EFS率为 (4 7.2± 12 .9) %。复发19例 (2 6 .0 % ) ,中位复发时间为 10个月 (2～ 5 3个月 )。死亡 2 8例。结论　AML XH 99方案进一步提高了儿童AML的CR率 ,尤其提高了 1个疗程CR率 ,是提高AML患儿EFS率的关键措施之一 ;HD Ara C作为儿童AML强化治疗方案是行之有效的。     

Levamisole in steroid-sensitive nephrotic syndrome children with frequent relapses and/or steroid dependency: comparison of daily and every-other-day usage

Steroid dependency (SD) and frequent relapses (FR) are common with steroid-sensitive nephrotic syndrome (SSNS). We assessed the effectiveness of daily levamisole in 36 children with SSNS with FR and/or SD. Twenty patients (group 1) were given levamisole 2-3 mg/kg q.o.d. for 4-24 months. Sixteen (group 2) had relapses within 3 months: 5 received levamisole q.d. for 3-18 months, and 11 q.d. for 6 months and then q.o.d. for 4-18 months. Follow-up was 4-36 (mean 20.4 +/- 9.2) months. After therapy, relapses (4.82 +/- 3.15 vs. 2.01 +/- 2.5 in group 1; 5.97 +/- 3.38 vs. 1.34 +/- 2.1 in group 2; p < 0.05) and prednisolone doses (0.57 +/- 0.37 vs. 0.15 +/- 0.33 mg/kg/day in group 1; 0.61 +/- 0.42 vs. 0.19 +/- 0.35 mg/kg/day in group 2; p < 0.05) decreased. Relapse frequency, prednisolone dose, response percentage, and survival curves for remission did not differ between groups. Group 1 had five episodes of leukopenia, and group 2 had four. White blood cell counts normalized after levamisole was discontinued. Serum blood urea nitrogen/creatinine and alanine aminotransferase/aspartate aminotransferase levels were normal. Levamisole is effective in maintaining remission in children with SSNS and FR and/or SD. Daily levamisole can be considered when responses to q.o.d. usage are unsatisfactory.