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Koga Y.,Japanese Pediatric Leukemia Lymphoma Study Group
[Rinshō ketsueki] The Japanese journal of clinical hematology | Year: 2012

Hodgkin lymphoma is an easily curable malignancy in the pediatric age group and is less frequently observed in Japan. No study with a large sample size of Japanese patients has been conducted. From 1985 to 2000, 157 Japanese patients with Hodgkin lymphoma were retrospectively analyzed based on their clinical characteristics, treatment regimen, and treatment outcome by 4 pediatiric cancer study groups. There were 107 male and 50 female patients with a median age of 10 years 1 month (range: 1 year 8 months to 17 years 8 months). Clinical stage I lymphoma was observed in 37 patients, stage II in 62, stage III in 40, and stage IV in 18. Fifty patients presented with B symptoms (32%). Most patients (n=125, 82%) received more than 6 courses of combination chemotherapy mainly comprising cyclophosphamide, vincristine, procarbazine, prednisolone (COPP), doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD). The 5-year overall and event-free survival rates were 81.5% and 94.8%, respectively. High-risk disease and age (>10 years) were considered to be poor prognostic factors. Source

Yanagimachi M.,Yokohama City University | Goto H.,Yokohama City University | Miyamae T.,Yokohama City University | Kadota K.,Yokohama City University | And 9 more authors.
Journal of Clinical Immunology | Year: 2011

Introduction Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome and has a varied genetic background. The polymorphism of interferon regulatory factor 5 gene (IRF5) was reported to be associated with susceptibility to macrophage activation syndrome. IRF5 acts as a master transcription factor in the activation of proinflammatory cytokines. We assessed associations of IRF5 gene polymorphisms with susceptibility to secondary HLH. Methods Three IRF5 single nucleotide polymorphisms (rs729302, rs2004640, and rs2280714) were genotyped using TaqMan assays in 82 secondary HLH patients and 188 control subjects. Results There was a significant association of the GT/TT genotype at rs2004640 with secondary HLH susceptibility (p<0.01). The IRF5 haplotype (rs729302 A, rs2004640 T, and rs2280714 T) was associated with secondary HLH susceptibility (p<0.01). Conclusions These findings indicate that IRF5 is a genetic factor influencing the susceptibility to secondary HLH and that the IRF5-associated immune response contributes to the pathogenesis of HLH. © 2011 Springer Science+Business Media, LLC. Source

Zwaan C.M.,Sophia Childrens Hospital Erasmus MC | Zwaan C.M.,International Berlin Frankfurt Munster Study Group I BFM SG New Agents Committee | Zwaan C.M.,Dutch Childhood Oncology Group | Kolb E.A.,Nemours Alfred I. du Pont Hospital for Children | And 35 more authors.
Journal of Clinical Oncology | Year: 2015

Diagnosis, treatment, response monitoring, and outcome of pediatric acute myeloid leukemia (AML) have made enormous progress during the past decades. Because AML is a rare type of childhood cancer, with an incidence of approximately seven occurrences per 1 million children annually, national and international collaborative efforts have evolved. This overview describes these efforts and includes a summary of the history and contributions of each of the main collaborative pediatric AML groups worldwide. The focus is on translational and clinical research, which includes past, current, and future clinical trials. Separate sections concern acute promyelocytic leukemia, myeloid leukemia of Down syndrome, and relapsed AML. A plethora of novel antileukemic agents that have emerged, including new classes of drugs, are summarized as well. Finally, an important aspect of the treatment of pediatric AML - supportive care - and late effects are discussed. The future is bright, with a wide range of emerging innovative therapies and with more and more international collaboration that ultimately aim to cure all children with AML, with fewer adverse effects and without late effects. © 2015 by American Society of Clinical Oncology. Source

Coenen E.A.,Erasmus University Rotterdam | Raimondi S.C.,St Jude Childrens Research Hospital | Raimondi S.C.,Childrens Oncology Group | Harbott J.,Justus Liebig University | And 41 more authors.
Blood | Year: 2011

We previously demonstrated that outcome of pediatric 11q23/MLL-rearranged AML depends on the translocation partner (TP). In this multicenter international study on 733 children with 11q23/MLL-rearranged AML, we further analyzed which additional cytogenetic aberrations (ACA) had prognostic significance. ACAs occurred in 344 (47%) of 733 and were associated with unfavorable outcome (5-year overall survival [OS] 47% vs 62%, P < .001). Trisomy 8, the most frequent specific ACA (n = 130/344, 38%), independently predicted favorable outcome within the ACAs group (OS 61% vs 39%, P = .003; Cox model for OS hazard ratio (HR) 0.54, P = .03), on the basis of reduced relapse rate (26% vs 49%, P < .001). Trisomy 19 (n = 37/344, 11%) independently predicted poor prognosis in ACAs cases, which was partly caused by refractory disease (remission rate 74% vs 89%, P = .04; OS 24% vs 50%, P < .001; HR 1.77, P = .01). Structural ACAs had independent adverse prognostic value for event-free survival (HR 1.36, P = .01). Complex karyotype, defined as ≥ 3 abnormalities, was present in 26% (n = 192/733) and showed worse outcome than those without complex karyotype (OS 45% vs 59%, P = .003) in univariate analysis only. In conclusion, like TP, specific ACAs have independent prognostic significance in pediatric 11q23/MLL-rearranged AML, and the mechanism underlying these prognostic differences should be studied. © 2011 by The American Society of Hematology. Source

Klein K.,VU University Amsterdam | Kaspers G.,VU University Amsterdam | Kaspers G.,Dutch Childhood Oncology Group | Harrison C.J.,Northumbria University | And 27 more authors.
Journal of Clinical Oncology | Year: 2015

Purpose: This retrospective cohort study aimed to determine the predictive relevance of clinical characteristics, additional cytogenetic aberrations, and cKIT and RAS mutations, as well as to evaluate whether specific treatment elements were associated with outcomes in pediatric t(8;21)-positive patients with acute myeloid leukemia (AML). Patients and Methods: Karyotypes of 916 pediatric patients with t(8;21)-AML were reviewed for the presence of additional cytogenetic aberrations, and 228 samples were screened for presence of cKIT and RAS mutations. Multivariable regression models were used to assess the relevance of anthracyclines, cytarabine, and etoposide during induction and overall treatment. End points were the probability of achieving complete remission, cumulative incidence of relapse (CIR), probability of event-free survival, and probability of overall survival. Results: Of 838 patients included in final analyses, 92% achieved complete remission. The 5-year overall survival, event-free survival, and CIR were 74%, 58%, and 26%, respectively. cKIT mutations and RAS mutations were not significantly associated with outcome. Patients with deletions of chromosome arm 9q [del(9q); n = 104] had a lower probability of complete remission (P = .01). Gain of chromosome 4 (+4; n = 21) was associated with inferior CIR and survival (P < .01). Anthracycline doses greater than 150 mg/m2 and etoposide doses greater than 500 mg/m2 in the first induction course and high-dose cytarabine 3 g/m2 during induction were associated with better outcomes on various end points. Cumulative doses of cytarabine greater than 30 g/m2 and etoposide greater than 1,500 mg/m2 were associated with lower CIR rates and better probability of event-free survival. Conclusion: Pediatric patients with t(8;21)-AML and additional del(9q) or additional +4 might not be considered at good risk. Patients with t(8;21)-AML likely benefit from protocols that have high doses of anthracyclines, etoposide, and cytarabine during induction, as well as from protocols comprising cumulative high doses of cytarabine and etoposide. © 2015 by American Society of Clinical Oncology. Source

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