儿童恶性淋巴瘤 60 例临床分析

目的探讨儿童恶性淋巴瘤的临床特征、疗效及预后的影响因素。方法回顾分析近10年收治的60例淋巴瘤患儿的临床资料,患儿按照WHO淋巴组织肿瘤分类标准和St.Jude分期系统进行分类、分期,并进行比较和分析。结果60例患儿中男43例、女17例,中位发病年龄8岁(6个月~15岁)。因局部肿物就诊者最多(21例,35.0%),骨髓及中枢神经系统受累比例分别为63.3%和6.7%。霍奇金淋巴瘤(HL)与非霍奇金淋巴瘤(NHL)分别占8.3%、91.7%。HL均为经典型霍奇金淋巴瘤,NHL常见类型包括淋巴母细胞性淋巴瘤、Burkitt淋巴瘤/白血病、间变性大细胞淋巴瘤及弥漫性大B细胞淋巴瘤。根据St.Jude分期,晚期(Ⅲ~Ⅳ期)57例,占95.0%;疾病进展/复发8例,发生事件中位时间为14个月(6~84个月)。患儿5年总生存率为(78±6)%,其中HL与NHL的5年总生存率分别为100%、(76±8)%,两组间差异无统计学意义(P=0.270)。COX分析显示,性别(P=0.038)是影响预后的独立因素。结论儿童淋巴瘤以男性多见,学龄期相对高发,就诊时多为晚期,以NHL多见。女性是影响预后的不良因素。     

儿童非核心结合因子急性髓系白血病的疗效及预后因素分析

目的 回顾性分析比较非核心结合因子（CBF）急性髓系白血病（AML）儿童采用CAMS-2005方案及CAMS-2009方案治疗的疗效及预后的影响因素。方法 选择2005年4月至2015年12月161例初诊为非CBF-AML患儿为研究对象，根据化疗方案分为CAMS-2005方案组（n=52）和CAMS-2009方案组（n=109），对两种化疗方案疗效进行回顾性分析。结果 CAMS-2009方案组第1个疗程完全缓解率高于CAMS-2005方案组（63.3% vs 46.2%，P < 0.05）。CAMS-2009方案组治疗相关病死率（11.9% vs 17.3%）、复发率（27.5% vs 28.8%）、3年总生存（OS）率（44%±5% vs 28%±6%）与CAMS-2005方案组相比差异无统计学意义（P > 0.05）。第1个疗程获得完全缓解患儿的3年OS率、3年无事件生存率高于第1个疗程未完全缓解患儿（P < 0.01）。结论 CAMS-2009方案较CAMS-2005方案可改善非CBF-AML患儿诱导治疗完全缓解率，第1个疗程是否获完全缓解可影响非CBF-AML患儿OS率。     

中晚期儿童肾透明细胞肉瘤10例的诊治及预后分析

目的探讨中晚期儿童肾透明细胞肉瘤(CCSK)的临床特点、诊治经过及预后特点。方法收集2014年1月至2017年12月在首都医科大学附属北京同仁医院儿科住院治疗的10例中晚期CCSK患儿的临床资料,对其临床特征、诊疗经过及预后情况等进行回顾性分析。结果1.临床特点:10例CCSK病例中男6例,女4例;中位发病年龄为32个月;7例为左侧CCSK,3例为右侧CCSK。初次诊断时Ⅲ期9例,Ⅳ期1例;其中4例初次诊断时误诊为肾脏其他肿瘤(40%,4/10例)。5例Ⅲ期病例治疗及随访过程中出现肿瘤复发及转移,主要远处转移部位为肺、骨、肝脏及脑。2.治疗及预后:10例中给予手术联合放疗及化疗者7例,未规范化治疗放弃者3例。中位随访时间33.5个月,7例存活,3例死亡,3年总生存率为65.6%。Ⅲ期患儿3年总生存率为74.1%,Ⅳ期患儿3年总生存率为0,Ⅲ期与Ⅳ期预后比较差异有统计学意义(χ^2=9,P=0.003)。5例复发病例中仅1例完全缓解,2例部分缓解,1例进展,1例死亡;3例无复发病例均完全缓解,且均为给予手术、化疗及放疗规范化治疗者。结论儿童CCSK初诊误诊率高,Ⅲ期病例治疗及随访过程中复发及远处转移风险高;Ⅲ期病例积极给予手术、化疗及放疗的规范化治疗,预后良好,而发生复发及远处转移者死亡率高。     

儿童急性巨核细胞白血病22例临床特征与预后分析

目的探讨儿童急性巨核细胞白血病的临床特征及预后影响因素。方法回顾性分析2011年2月至2017年8月收治的22例急性巨核细胞白血病患儿的临床资料,生存分析采用Kaplan-Meier法,行Log-Rank检验。结果 22例患儿的中位随访时间10.8个月。其中4例患儿诊断后放弃治疗,3例在第1疗程化疗过程中放弃治疗,余15例1、2疗程的完全缓解率为66.7%和93.3%,8例患儿随访期间复发。22例患儿2年总生存率、无事件生存率分别为(36.4±13.7)%和(22.2±10.7)%。单因素分析显示,伴有21三体组的2年总生存率高于不伴有21三体组;染色体数目为49～60条组的2年无事件生存率高于其他数目组;初诊白细胞计数10×109/L组的2年总生存率及无事件生存率高于≥10×109/L组;第1次骨髓完全缓解后行异基因造血干细胞移植组患儿的2年总生存率及无事件生存率高于单纯化疗组,差异均有统计学意义(P0.05)。结论儿童急性巨核细胞白血病是一类具有高度异质性的疾病,复发率高,预后差。初诊白细胞计数及染色体核型是影响预后的重要因素,骨髓完全缓解后应尽快行异基因造血干细胞移植。     

CCLG-ALL2008方案治疗儿童急性淋巴性白血病单中心疗效分析

目的分析CCLG-ALL2008方案治疗儿童急性淋巴细胞白血病(ALL)的单中心疗效,为改进该方案提供临床依据。方法符合入组标准的100例ALL患儿,接受了CCLG-ALL 2008方案治疗,回顾性分析治疗结果及治疗相关毒副作用。结果 100例中低危ALL 49例,中危23例,高危28例。诱导缓解率97%。化疗期间发生严重感染24例(24%),并发大脑后部可逆性脑病综合征(PRES)6例,发生治疗相关死亡(TRM)8例。本组病人的2年和5年累积复发率均为(12%±0.04),2年和5年总体生存率(0S)均为(83%±0.04),2年和5年无事件生存率(EFS)均为(79%±0.04)。低危组与高危组间0S差异有显著性(χ~2=12.026,P=0.001);低危组与高危组间EFS差异有显著性(χ~2=14.291,P=0.000),中危组与高危组间EFS差异亦有显著性(χ~2=5.356,P=0.021)。影响生存的主要因素是诱导期严重感染所致TRM、疾病复发以及病人治疗途中失访。结论 CCLG-ALL 2008方案治疗儿童ALL完全缓解率高、复发率低,降低诱导期严重感染所致TRM,减少高危疾病复发,并改进高危病人综合管理以使病人能接受完整治疗,可进一步提高生存率。     

急性粒细胞性白血病的骨髓移植

在急性粒细胞性白血病(AML)的治疗中,骨髓移植是否优于化学疗法尚无定论。多数报告认为AML的首次缓解期中,骨髓移植的疗效以年轻患者为佳。根据国际骨髓移植中心发表的资料,1973～1986年于首次缓解期中施行骨髓移植的704例AML的5年生存率为48%,白血病的复发率为20%。从年龄上来看,年龄愈轻生存率愈高,25岁以下非白血病的5年生存率为58%,25岁以上为38%。文献中有关AML的治疗采用骨髓移植优于化学疗法的报告很多。Conde等报告AML的首次缓解期中,骨髓移植组3年的完全缓解率为70%,而化学疗法为10%,显示很大的差异。从年龄差别上来看,Weisdorf等报告149例白血病,移植后3年内的完全缓解率,18岁以上的48例中为45.4%,不满18岁的101例的完全缓解率为39.9%。AML骨髓移植后3年内的完全缓解率,成人为51.60%,小儿为52.9%,未见年龄差异。     

儿童混合表型急性白血病15例临床特征分析北大核心CSCD

目的分析儿童混合表型急性白血病(MPAL)的临床特点、治疗及预后, 为临床优化诊疗方案及提高缓解率提供参考。方法基于2016年世界卫生组织(WHO)的诊断标准, 回顾性分析2012年1月至2020年12月苏州大学附属儿童医院收治的15例MPAL患儿的骨髓细胞形态、免疫分型、细胞遗传学、分子生物学特征以及治疗方案、预后等病例资料。计数数据组间比较采用χ^(2)检验, 符合正态分布的计量资料组间比较采用t检验, 非正态分布的计量资料组间比较采用秩和检验。采用Kaplan-Meier(K-M)法估计生存率, 比较应用Log-rank法。结果苏州大学附属儿童医院8年共收治15例MPAL患儿, 男8例, 女7例, 中位年龄为6.8岁;9例患儿表达B淋系+髓系表型, 5例表达T淋系+髓系表型, 1例表达B淋系+T淋系表型;11例患儿进行了染色体核型检查, 2例为正常核型, 2例为复杂核型, 6例为假二倍体, 1例为亚二倍体;5例患儿检测到融合基因, 其中3例AML-ETO阳性, 1例BCR-ABL阳性, 1例MLL阳性;13例患儿在化疗后完全缓解, 总完全缓解率为86.6%, 2年总生存率为(68.2±13.4)%。15例患儿中14例授受了诱导化疗, 1例因个人原因放弃了治疗。首选急性淋巴细胞白血病(ALL)化疗方案10例, 第1个疗程完全缓解1例, 总完全缓解率10%;首选急性髓系白血病(AML)化疗方案4例, 第1个疗程完全缓解3例, 总完全缓解率75%, 未缓解的1例更换ALL方案后缓解;8例行造血干细胞移植(HSCT)和6例未行HSCT组2年总生存率分别为(70.0±18.2)%、(66.7±19.2)%, 差异无统计学意义(χ^(2)=0.318, P=0.573)。结论儿童MPAL是一种罕见的恶性肿瘤, 以淋系和髓系抗原共表达为主, 单纯化疗或HSCT在短期内均可获得较好的预后, 但长期疗效还有待进一步观察。     

儿童急性T淋巴细胞白血病的临床特征及预后分析

目的 分析儿童急性T淋巴细胞白血病(T-ALL)的临床特征、预后因素及生存情况。方法 回顾性分析2008年4月—2020年8月于首都儿科研究所附属儿童医院血液内科确诊的84例T-ALL临床和实验室资料。并将非早前T淋巴细胞(non-ETP)-ALL组与早前T淋巴细胞(ETP)-ALL组进行比较。结果 non-ETP组共70例(83.3%),ETP组共14例(16.7%)。与non-ETP组相比,ETP组女性比例高、LDH明显增高者比例低、高危者比例高、诱导失败者比例高(P<0.05)。non-ETP组完全缓解(CR)率为87.1%,ETP-ALL组CR率为57.1%。non-ETP组复发19例,死亡15例;ETP组复发3例,死亡3例。non-ETP-ALL及ETP-ALL组的5年的总生存率(OS)分别为：(77.1±5.3)%vs.(74.5±13.1)%(P=0.979)。两组患儿5年的无事件生存率(EFS)分别为：(64.2±6.0)%vs.(50±13.4)%(P=0.098)。通过Kaplan-Meier分析进行单因素分析显示,诱导失败及复发为影响OS的因素(P<0...     

NPCLC-ALL2008方案治疗儿童急性T淋巴细胞白血病的生存分析

目的探讨中国儿童急性淋巴细胞白血病2008(NPCLC-ALL2008)方案治疗儿童急性T淋巴细胞白血病(T-ALL)的效果。方法回顾性分析2009年1月至2017年12月在浙江大学医学院附属儿童医院血液肿瘤中心接受NPCLC-ALL2008方案进行规范治疗的96例T-ALL患儿的临床资料。分析流式细胞术监测微小残留病(MRD)与预后的关系,应用Kaplan-Meier法进行长期生存分析。结果 96例T-ALL患儿中男72例、女24例,年龄9.5(1.0～16.0)岁,随访时间5.7(1.0～9.7)年。92例患儿获得完全缓解(96%),5年无事件生存率(EFS)和总生存率(OS)分别为(61±6)%和(70±5)%,复发18例,5年累积复发率(27±6)%,死亡24例。诱导第15天MRD>5%者生存率明显低于MRD≤5%者,5年OS分别为(60±12)%和(72±6)%(χ2=3.904,P=0.048),巩固治疗前MRD>10%者5年EFS和OS均明显降低,均为(50±35)%。复发患儿生存明显差于无复发患儿,5年OS分别为(26±13)%和(81±5)%(χ2=18....     

大剂量化疗结合自体外周血干细胞移植治疗神经外胚层来源的儿童实体瘤疗效分析

目的：探讨大剂量化疗结合自体外周血造血干细胞移植（APBSCT）治疗神经外胚层来源晚期实体瘤的疗效。方法：选择神经外胚层来源的肿瘤患儿23例,平均年龄5.8±3.5岁。移植前化疗8.0±4.3疗程。其中IV期神经母细胞瘤20例,移植时完全缓解9例,部分缓解7例,处于肿瘤进展期4例;原始神经外胚叶肿瘤2例,移植期间为完全缓解。视网膜母细胞瘤1例,移植时部分缓解。进行外周血造血干细胞采集、手术切除,然后进行APBSCT。结果：①20例神经母细胞瘤患儿中,19例移植后造血功能获得满意重建,重建时间为16.5±0.9 d。术后中位随访时间为15.8月。移植前获完全缓解的患儿生存率为100%,部分缓解患儿为57%,进展期患儿为0（P<0.05）。3组15.8月总生存率67%。②视网膜母细胞瘤患儿随访6个月获完全缓解。③原始神经外胚叶肿瘤患儿于术后5~8个月原发灶复发,1年内均死亡。结论：大剂量化疗结合APBSCT治疗神经外胚层来源实体瘤,在移植前达到完全缓解者可取得较好疗效,部分缓解者可提高缓解率。其中原始神经外胚叶肿瘤预后最差,即使在完全缓解时移植死亡率仍高。［中国当代儿科杂志,2010,12（4）：244-247］     

Childhood NHL in Switzerland: Incidence and Survival of 120 Study and 42 Non-Study Patients

Based on the Swiss Pediatric Oncology Group (SPOG) cancer registry data during 1981–1991, a high average incidence of 8 new NHL per million children younger than 15 years per year was found. Of 162 children with NHL registered in 1976–1991, 120 were study patients, i.e., officially registered and treated according to SPOG or Pediatric Oncology Group (POG) protocols, while 42 were non-study patients, i.e., patients not officially enrolled on protocols. Overall, 91 of 120 (76%) study patients remained alive. Seventy-nine study patients were treated according to older SPOG protocols, and 53 (67%) of these survived, while 38 of 41 (93%) study patients treated according to newer POG protocols remained alive (P = 0.0068). Only 22 (52%) of the 42 non-study patients survived (P = 0.0001). There was no improvement if the survival of non-study patients before and since 1986 was compared. Population-based treatment results in Switzerland were similar to those in the United Kingdom. They provided an important base for the development of future treatment strategies. © 1995 Wiley-Liss, Inc.     

Acute megakaryoblastic leukemia (excluding Down syndrome) remains an acute myeloid subgroup with inferior outcome in the French ELAM02 trial

We report the outcome of 27 children with de novo acute megakaryoblastic leukemia (AMKL) (excluding Down syndrome) enrolled in the French multicenter prospective study ELAM02 (2005-2011). There was no difference in gender, initial leukocyte count, CNS involvement, and complete remission rate (88.9%), as compared to other acute myeloid leukemia (AML) subtypes. AMKL patients had a significantly poorer outcome (5-year overall survival 54% [CI 95% 33%–71%] than children with other AML subtypes (5-year overall survival 73% [CI 95% 68%–77%] p = 0.02). Gender, age, CNS leukemia, hyperleukocytosis, complete remission or cytogenetic subgroups were not significant prognostic factors of disease-free survival. AMKL (excluding Down syndrom) remains an AML subgroup with inferior outcome.     

High-grade astrocytoma in very young children

BACKGROUND: High-grade astrocytomas are rare in young children, but have been reported to have a better prognosis than similar tumors in older patients. PROCEDURE: We retrospectively reviewed the clinical characteristics, survival, and long-term sequelae for patients younger than 3 years old with high-grade astrocytoma, treated at a single institution between 1984 and 2005. RESULTS: Sixteen patients were included. Histology included anaplastic astrocytoma (n = 9), glioblastoma multiforme (n = 5), and malignant glioma (n = 2). All patients underwent biopsy or resection, followed by chemotherapy. Six patients received scheduled irradiation and six were irradiated at the time of disease progression. Ten patients are alive at a median follow-up of 11.6 years (range, 1.7-21.6 years). 5-year overall survival (OS) was 66.3% (SE 12.2%), and 5-year event-free survival (EFS) was 28.6% (SE 12.1%). Age at diagnosis was a significant predictor of the hazard of death in a Cox model (HR 2.871, 95%CI 1.015-8.123). Gender and histology did not predict OS or EFS. Trends toward improved OS were detected for patients with hemispheric tumors and those undergoing complete resection. All evaluable survivors (n = 9) had some neurocognitive impairment, with estimated overall cognitive ability ranging from significantly delayed to average; all survivors attending school (n = 5) performed below grade level on achievement testing. Seven of nine evaluable survivors had endocrine dysfunction. CONCLUSIONS: Young children with high-grade astrocytoma have better long-term overall survival than older patients, but recurrence is common, and most children require irradiation. Long-term complications are frequent and often severe.     

Risk of recurrence and survival after relapse in patients with Ewing sarcoma

Background

The prognosis in patients with relapsed Ewing sarcoma is unfavorable. Our investigation identifies factors predicting for the outcome following relapse.

Procedure

We analyzed type of relapse, time to relapse and overall survival after relapse (OSr) in 714 patients with first recurrence. All patients had been treated within the Cooperative Ewing Sarcoma Studies (CESS) 81 or 86, or the European Intergroup CESS (EICESS 92). OSr time was calculated from diagnosis of first relapse to last follow‐up or death.

Results

Median follow‐up time from diagnosis of primary disease was 2.2 years (mean = 4.0; range: 0.2–24.9). Relapse sites were local in 15%, combined local and systemic in 12%, and systemic in 73%. Among patients with a localized primary tumor, 20% relapsed locally, while 12% showed combined and 68% systemic relapse. When the primary disease was disseminated, 82% developed systemic, 13% combined, and 5% local relapse. Five‐year OSr was 0.13 (SE = 0.01). Outcome following local relapse, with a 5‐year survival rate of 0.24 (P < 0.001), was superior to outcome after systemic or combined recurrence. Five‐year OSr was 0.07 (SE = 0.01) in patients who relapsed 0–2 years after the diagnosis of primary disease, as compared to a 5‐year OSr of 0.29 (SE = 0.03) when relapse occurred later.

Conclusions

5‐year OSr in Ewing sarcoma is poor (<0.2). Prognostically favorable factors are: late onset (>2 years) and strictly localized relapse. Pediatr Blood Cancer 2011; 57: 549–553. © 2011 Wiley‐Liss, Inc.     

Successful treatment of multiply relapsed lymphoma with rituximab as a single agent

The treatment of non‐Hodgkin lymphoma (NHL) is an example of the successful therapy of cancer in children; the cure rate overall at approximately 80%. Unfortunately, relapsed NHL has a dismal prognosis, and the customary treatment is highly toxic chemotherapy followed by hematopoietic stem cell transplantation (HSCT). A child with relapsed T‐cell rich B‐NHL was treated with rituximab alone. This was delivered in eight doses after the recurrence of disease after high‐dose chemotherapy and allogenic HSCT that was undertaken for the first relapse. Eight years after the last dose of rituximab he remains in complete remission. Pediatr Blood Cancer. 2010;55:356–358. © 2010 Wiley–Liss, Inc.     

Intracranial relapse in Wilms tumor patients

BACKGROUND: In children with nephroblastoma, recurrence with metastases in the central nervous system is rare. Recently, previous reports (NWTSG and UKCCSG) reported brain metastases with an incidence of respectively 0.5% and 0.6% in Wilms tumor (WT) patients (respectively n = 30/5,852 and n = 7/1,249). PROCEDURE: We retrospectively investigated the incidence and survival of patients with central nervous system relapse in WT patients, treated according to the consecutive SIOP protocols 1, 2, 5, 6, 9, and 93-01. All children with WT from 1971 until 2000 were enrolled in the study (3,040 eligible patients). Specimens at diagnosis and if possible at relapse were centrally reviewed. Patients with renal neoplasms other than WT were excluded. RESULTS: CNS relapse was documented in 14 patients (0.5%). Median time to CNS relapse was 16 months (3-69). The occurrence of relapse was not associated with specific histological subtypes. In seven patients intracranial metastases occurred at first relapse, of which two were isolated relapses. In five patients no treatment was started because of the poor condition of the patient, the other nine cases were treated with (a combination of) chemotherapy (n = 6), surgery (n = 4), and radiotherapy (n = 6). CONCLUSIONS: CNS relapse in WNT is rare. In contrast to reports of other Wilms tumor study groups, although four patients reached (local) CR, the SIOP registry showed that eventually none of the documented WT patients survived.     

Intensive chemotherapy without radiotherapy gives more than 85% event-free survival for non-Hodgkin lymphoma without central nervous involvement: a 6-year population-based study from the nordic society of pediatric hematology and oncology

BACKGROUND: The prognosis in childhood non-Hodgkin lymphoma (NHL) has improved dramatically during recent decades. The authors report the results from a 6-year population-based study of clinical characteristics and treatment results of NHL from the five Nordic countries. METHODS: All children younger than 15 years of age at diagnosis with NHL diagnosed from 1995 to 2000 were stratified and treated according to immunophenotypic classification and stage of disease. RESULTS: A total of 230 patients were diagnosed with primary NHL, which gives an annual incidence of 0.9/100.000 children, with a median age of 8 years. Seven percent of the children were below 3 years of age at diagnosis. The male/female ratio was 2.3 and was unrelated to age. Patients with pre-B and T-cell NHL constituted 33%, B-cell NHL 53%, and anaplastic large cell lymphoma (ALCL) 14%. According to Murphy's classification, 14% had stage 1, 17% stage 2, 50% stage 3, and 19% stage 4 disease, 12 of whom (28%) had central nervous involvement (CNS) at diagnosis. By January 1, 2003, four children had died during induction, three children died in remission (2, 6, and 26 months from diagnosis), and 24 children experienced a relapse. At 5 years, the probability of event-free survival (p-EFS) was 86+/-2% for all children. The 5-year p-EFS values for stages 1 through 4 were 94%, 97%, 83%, and 79%, respectively. The 5-year p-EFS values were 91% for B-cell, 87% for pre-B, 81% for ALCL, and 79% for T-cell NHL. The 12 patients with CNS involvement at diagnosis had a significantly poorer outcome than stage 4 patients with CNS involvement (p-EFS = 50% vs. 90%, P < 0.01). The 218 patients without CNS disease at diagnosis had a 5-year p-EFS of 88%. CONCLUSIONS: With modern intensive chemotherapy, more than 85% of NHL patients will achieve long-lasting first remission. In the future, preventing death during induction and remission and improving therapy for patients with CNS disease would have a major impact on the overall p-EFS.     

Treatment of children with B-cell non-Hodgkin's lymphoma in developing countries: the experience of a single center in Brazil

PURPOSE: To treat non-Hodgkin's B-cell lymphoma (B-NHL) in children with manageable toxicity-related morbidity and without any decrease in survival. PATIENTS AND METHODS: Between January 1998 and April 2003, 53 consecutive patients (age 16 years or less) from a single institution were enrolled. The patients were stratified by risk factors (stage and LDH level) and treated with a BFM 86/90 (Berlin-Frankfurt-Münster)-based protocol with reduction of the methotrexate dose from 5 mg/m to 2 mg/m. RESULTS: The mean age of the patients was 6 years (range 1-16 years). Seventy-two percent of the patients had lymphomas classified as Burkitt type, 11% as diffuse large cell lymphoma, and 6% as Burkitt-like lymphoma, and 11% were not classified. At a median follow-up of 35 months, 44 patients (83%) survived in complete remission. The event-free survival rate for all patients was 78% (SE = 0.07): 100% (SE = 0.0) for stage I/II patients and 74% (SE = 0.08) for stage III/IV patients. Six patients suffered initial treatment failure and one patient relapsed, all of whom died. There was only one death from sepsis related to treatment. CONCLUSIONS: This strategy was very effective for treating B-NHL in a developing country. The results were comparable to those of the BFM 90 study and other contemporary groups and represented an increase in the cure rates in childhood B-NHL in Brazil.     

Non-Hodgkin lymphoma in children: a 20-year population-based epidemiologic study in western Sweden

PURPOSE: The aim of this study was to investigate incidence, clinicopathologic features, prognostic risk factors, and long-term survival in non-Hodgkin lymphoma (NHL) in a 20-year population-based study of children using Swedish health care organizations and their central registry for childhood malignancies. PATIENTS AND METHODS: The hospital registry, the Cause of Death Registry, and the two established Swedish registries for malignancy (the Swedish Cancer Registry and the National Registry for Solid Tumours in Childhood) were searched for children in western Sweden with NHL diagnosed from 1975 to 1994. The clinical files of all children with NHL were collected and abstracted for information regarding age at diagnosis, gender, disease characteristics, treatment, and outcome of treatment. All sections from paraffin-embedded blocks of tumors with a diagnosis of malignant lymphoma were collected and reexamined histopathologically and immunohistochemically. To guarantee that no patients with NHL were misdiagnosed, a reexamination of other childhood malignancies collected from these registries was also performed. Median follow-up duration of surviving patients is 10 years. RESULTS: The annual incidence of NHL in children younger than 15 years of age was 9/million children, representing 6% of all childhood malignancies during the investigation time. The male-female ratio was 4.1:1.0. Immunologic marker studies were available for 64 of the 77 NHLs: 41 patients had B-cell, 17 had T-cell, and 6 had Ki-1-positive anaplastic large cell lymphoma (ALCL). Two patients with Ki-1-positive ALCL were originally thought to have malignant histiocytosis and Langerhans cell histiocytosis (LCH), respectively. Treatment was the most significant prognostic factor; event-free survival (EFS) was 19% in the preprotocol era (1975 to 1979) and 74% from 1980 to 1994. Other than treatment, stage was the most significant prognostic factor; EFS was 86% for patients (1980 to 1994) with stage I or II disease and 64% for patients with stage III or IV disease, with a dismal prognosis for children with initial involvement of the bone marrow or central nervous system (EFS was 38% and 20%, respectively). Bulky disease and performance state at diagnosis were independent prognostic factors. The patterns of relapse, including early recurrence of the B-cell lymphomas, are in accordance with previous experience. CONCLUSION: The incidence of NHL was found to be somewhat higher than reported in our previous Nordic study. The higher incidence found in this study might be the result of the thorough data collection (based on hospital registry and cross-checked with all registries for malignant diseases in Sweden) or because reexamination of the tissue material was performed. A more pronounced male predominance than found in previous investigations was observed. The immunophenotypic distribution and the stage distribution is in accordance with earlier investigations. Treatment was the most important factor affecting outcome. A dramatic improvement of survival was seen with the introduction of intensive therapy; treatment success can be expected in 86% of children with localized disease and 64% of children with extensive disease. The absence of improvement in survival despite further treatment stratification with the introduction of the BFM protocol for B-cell-NHL is surprising. LSA2L2-like protocols seem to be as effective. Future studies on treatment of NHL must also concentrate on reducing the intensity of therapy in patients with lower risk disease to minimize late toxic effects.     

The outcome of hematopoietic stem cell transplantation in Korean children with hemophagocytic lymphohistiocytosis

Yoon HS, Im HJ, Moon HN, Lee JH, Kim H‐J, Yoo KH, Sung KW, Koo HH, Kang HJ, Shin HY, Ahn HS, Cho B, Kim HK, Lyu CJ, Lee MJ, Kook H, Hwang TJ, Seo JJ. The outcome of hematopoietic stem cell transplantation in Korean children with hemophagocytic lymphohistiocytosis.
Pediatr Transplantation 2010: 14:735–740. © 2010 John Wiley & Sons A/S. Abstract: Chemoimmunotherapy‐based treatments have improved the survival of patients with HLH, but outcomes of the patients are still unsatisfactory. We report here the outcome of Korean children with HLH who underwent HSCT, which was analyzed from the data of a nation‐wide HLH registry. Retrospective nation‐wide data recruitment for the pediatric HLH patients diagnosed between 1996 and 2008 was carried out by the Histiocytosis Working Party of the Korean Society of Hematology. Nineteen patients who received HSCT among the total of 148 enrolled children with HLH were analyzed for the transplant‐related variables and events. The probability of five‐yr survival after HSCT was 73.3% with a median follow‐up of 57. Two months compared to 54.3% for the patients who were treated with chemoimmunotherapy only (p = 0.05). The reasons for HSCT were active disease after eight wk of initial treatment (n = 9), relapsed disease (n = 5), and FHL (n = 5). Fourteen patients are currently alive without disease after HSCT, four patients died of treatment‐related events (infection in two and graft failure in two) at early post‐transplant period, and one patient died of relapse at one yr post transplantation. The survival of patients who were transplanted because of active disease after eight wk of initial treatment was worse compared to those patients who had inactive state at that time (60.6% vs. 100%, respectively, p = 0.06). Of the four patients who received transplants using cord blood, three died of graft failure (n = 2) and relapse (n = 1). The five‐yr probability of survival after HSCT according to the donor type was 85.7% for the MRDs (n = 6), 87.5% for the MUDs (n = 8), and 40% for the MMUDs (n = 5) (p = 0.03). Other variables such as age, CNS involvement at the time of diagnosis, the etiology of HLH (familial or secondary), and the conditioning regimens had no influence on the five‐yr OS of the HLH patients who underwent HSCT. HSCT improved the survival of the patients who had familial, relapsed, or severe and persistent SHLH in the Korean nation‐wide HLH registry. Although numbers were small, these results are similar to other reports in the literature. The disease state after initial treatment, the stem cell source of the transplant, and the donor type were the important prognostic factors that affected the OS of the HLH patients who underwent HSCT.