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https://www.readbyqxmd.com/read/28333413/extramedullary-leukemia-in-children-with-acute-myeloid-leukemia-a-population-based-cohort-study-from-the-nordic-society-of-pediatric-hematology-and-oncology-nopho
#1
Heidi Kristine Støve, Julie Damgaard Sandahl, Jonas Abrahamsson, Peter H Asdahl, Erik Forestier, Shau-Yin Ha, Kirsi Jahnukainen, Ólafur G Jónsson, Birgitte Lausen, Josefine Palle, Bernward Zeller, Henrik Hasle
BACKGROUND: The prognostic significance of extramedullary leukemia (EML) in childhood acute myeloid leukemia is not clarified. PROCEDURE: This population-based study included 315 children from the NOPHO-AML 2004 trial. RESULTS: At diagnosis, 73 (23%) patients had EML: 39 (12%) had myeloid sarcoma, 22 (7%) had central nervous system disease, and 12 (4%) had both. EML was associated with young age (median age: 2.6 years), a high white blood cell count (median: 40 × 10(9) /l), M5 morphology (40%), and 11q23/MLL (KMT2A) rearrangements (34%)...
March 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28284743/whole-body-mr-imaging-a-useful-imaging-modality-in-the-management-of-children-with-acute-myeloid-leukemia
#2
Hee Mang Yoon, Jeong Rye Kim, Ah Young Jung, Young Ah Cho, Ho Joon Im, Jin Seong Lee
INTRODUCTION: To evaluate the distribution of chloromas using whole body magnetic resonance (MR) imaging in pediatric patients with acute myeloid leukemia (AML) and to assess the clinical role of whole body MR imaging in management of pediatric patients with AML. MATERIALS AND METHODS: We retrospectively searched pediatric patients (< 18 years old) who were diagnosed with AML and underwent whole body MR imaging during their illness between January 2006 and December 2014...
February 16, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28278270/methylation-level-of-cpg-islands-in-ggh-gene-promoter-in-pediatric-acute-leukemia
#3
Yue Li, Sixi Liu, Huihui Wang, Huirong Mai, Xiuli Yuan, Changgang Li, Xiaowen Chen, Feiqiu Wen
BACKGROUND: γ-Glutamyl hydrolase (GGH) regulates intracellular folates and antifolates such as methotrexate (MTX) for proper nucleotide biosynthesis and antifolate-induced cytotoxicity, respectively. In addition to genetic polymorphism and karyotypic abnormalities, methylation of CpG island 1 (CpG1) in the promoter region is found to modulate GGH activity by reducing GGH mRNA expression in acute lymphoblastic leukemia (ALL) cells. We aim to investigate methylation status of two CpG islands (CpG1 and CpG2) in the GGH promoter region in pediatric patients with ALL and acute myelogenous leukemia (AML)...
2017: PloS One
https://www.readbyqxmd.com/read/28266766/early-mixed-t-cell-chimerism-is-predictive-of-pediatric-aml-or-mds-relapse-after-hematopoietic-stem-cell-transplant
#4
Larisa Broglie, Irene Helenowski, Lawrence J Jennings, Kristian Schafernak, Reggie Duerst, Jennifer Schneiderman, William Tse, Morris Kletzel, Sonali Chaudhury
Patients with acute myeloid leukemia (AML) who relapse after hematopoietic stem cell transplantation (HCT) have dismal outcomes. Our ability to predict those at risk for relapse is limited. We examined chimerism trends post-HCT in 63 children who underwent HCT for AML or myelodysplastic syndrome (MDS). Mixed T-cell chimerism at engraftment and absence of chronic graft versus host disease (cGVHD) were associated with relapse (P = 0.04 and P = 0.02, respectively). Mixed T-cell chimerism at engraftment was predictive in patients without cGVHD (P = 0...
March 7, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28253492/identification-of-a-cryptic-insertion-ins-11-x-q23-q28q12-resulting-in-a-kmt2a-flna-fusion-in-a-13-month-old-child-with-acute-myelomonocytic-leukemia
#5
Jana Lentes, Kathrin Thomay, Dominik T Schneider, Benedikt Bernbeck, Dirk Reinhardt, Rolf Marschalek, Claus Meyer, Brigitte Schlegelberger, Gudrun Göhring
In pediatric acute myeloid leukemia (AML), chromosomal abnormalities leading to a disruption of the lysine methyltransferase 2A (KMT2A) gene in 11q23 are the most frequent rearrangements. Here, we report on the identification of a novel cryptic insertion, ins(11;X)(q23;q28q12), resulting in a translocation of the KMT2A gene in 11q23, leading to a KMT2A-FLNA fusion in a 13-month-old boy with de novo acute myelomonocytic leukemia, who died 38 days after diagnosis. The patient presented a complex karyotype 48∼49,Y,del(X)(q12),+del(X)(q12),+8,ins(11;X)(q23; q28q12),+19...
March 3, 2017: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/28218757/allogeneic-stem-cell-transplantation-for-refractory-acute-myeloid-leukemia-in-pediatric-patients-the-uk-experience
#6
P O'Hare, G Lucchini, M Cummins, P Veys, M Potter, S Lawson, A Vora, R Wynn, A Peniket, K Kirkland, R Pearce, J Perry, P J Amrolia
We report outcomes for 44 children who underwent stem cell transplantation (SCT) for refractory AML in the UK between 2000 and 2012. Median age at SCT was 11.5 years. Twenty-three patients had primary refractory and 21 relapsed refractory AML. Refractory disease was confirmed by cytogenetics/molecular genetics in 24 cases. Median follow-up of the whole cohort is 6.8 years (2.1-14.9 years). Thirty patients (68%) achieved a CR following SCT. Transplant-related mortality at 1 year was 18%. Acute GVHD incidence was 52% (grade ⩾III 19%), chronic 7%...
February 20, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28212370/the-evolution-of-allogeneic-stem-cell-transplant-for-children-and-adolescents-with-acute-myeloid-leukemia
#7
Allyson Flower, Mitchell S Cairo
Survival rates in subsets of pediatric patients who have acute myeloid leukemia (AML) with favorable risk features are now greater than 90%. However, outcomes for patients with high-risk (HR) features remain unacceptably poor. As novel technologies for the identification of HR biomarkers and the detection of residual disease are developed, risk stratification and the application of allogeneic hematopoietic stem cell transplant (HSCT) are evolving. HSCT has been shown to benefit subpopulations of pediatric patients with AML, including those with HR cytogenetic translocations, genetic mutations, and/or residual disease after induction...
January 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/28197035/giant-renal-angiomyolipoma-masquerading-as-a-wilms-tumor
#8
Anjan Kumar Dhua, Abhishek Ranjan, Sandeep Agarwala, Veereshwar Bhatnagar, Sandeep R Mathur, Kandasamy Devasenathipathy
Renal Angiomyolipoma (AML) is not commonly seen in the pediatric age group other than patients of tuberous sclerosis where in they can have renal AMLs within the first decade with bilateral in involvement. Diagnosis of renal AML can generally be made by the current radiological modalities in the appropriate clinical setting, but it can be mistaken for other tumors when it is large and has low-fat content. Herein we report a case of giant renal AML that was initially misdiagnosed as a Wilms tumor in a 12-year-old girl...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28144203/socio-demographic-characteristics-of-patients-with-diagnosis-of-leukemia-in-bosnia-and-herzegovina-during-six-year-period
#9
Edo Hasanbegovic, Nedim Begic, Snijezana Hasanbegovic, Zijo Begic
INTRODUCTION: Malignancies in children are second most frequent cause of death after accidents in pediatric age, while leukemias represents 30% of all malignancies of children age. AIM: To present the social and demographic characteristics of patients with a diagnosis of leukemia in Bosnia and Herzegovina during six years period. PATIENTS AND METHODS: The study included 97 patients (from birth to 18 years old) with diagnosis of acute leukemia...
December 2016: Materia Socio-medica
https://www.readbyqxmd.com/read/28123582/ets-related-gene-is-a-novel-prognostic-factor-in-childhood-acute-lymphoblastic-leukemia
#10
Hai-Zhao Zhao, Ming Jia, Ze-Bin Luo, Xiao-Jun Xu, Si-Si Li, Jing-Ying Zhang, Xiao-Ping Guo, Yong-Min Tang
The ETS-related gene (ERG) has been demonstrated to be associated with overall survival in cytogenetically normal acute myeloid leukemia and acute T cell-lymphoblastic leukemia (T-ALL) in adult patients. However, there are no data available regarding the impact of ERG expression on childhood ALL. In the present study, ERG expression levels were analyzed in bone marrow samples from 119 ALL pediatric patients. ALL patients demonstrated higher ERG expression compared with the controls (P<0.0001). In addition, low ERG expression identified a group of patients with higher white blood cell counts (P=0...
January 2017: Oncology Letters
https://www.readbyqxmd.com/read/28112737/pediatric-non-down-syndrome-acute-megakaryoblastic-leukemia-is-characterized-by-distinct-genomic-subsets-with-varying-outcomes
#11
Jasmijn D E de Rooij, Cristyn Branstetter, Jing Ma, Yongjin Li, Michael P Walsh, Jinjun Cheng, Askar Obulkasim, Jinjun Dang, John Easton, Lonneke J Verboon, Heather L Mulder, Martin Zimmermann, Cary Koss, Pankaj Gupta, Michael Edmonson, Michael Rusch, Joshua Yew Suang Lim, Katarina Reinhardt, Martina Pigazzi, Guangchun Song, Allen Eng Juh Yeoh, Lee-Yung Shih, Der-Cherng Liang, Stephanie Halene, Diane S Krause, Jinghui Zhang, James R Downing, Franco Locatelli, Dirk Reinhardt, Marry M van den Heuvel-Eibrink, C Michel Zwaan, Maarten Fornerod, Tanja A Gruber
Acute megakaryoblastic leukemia (AMKL) is a subtype of acute myeloid leukemia (AML) in which cells morphologically resemble abnormal megakaryoblasts. While rare in adults, AMKL accounts for 4-15% of newly diagnosed childhood AML cases. AMKL in individuals without Down syndrome (non-DS-AMKL) is frequently associated with poor clinical outcomes. Previous efforts have identified chimeric oncogenes in a substantial number of non-DS-AMKL cases, including RBM15-MKL1, CBFA2T3-GLIS2, KMT2A gene rearrangements, and NUP98-KDM5A...
March 2017: Nature Genetics
https://www.readbyqxmd.com/read/28111878/first-experience-of-the-aml-berlin-frankfurt-m%C3%A3-nster-group-in-pediatric-patients-with-standard-risk-acute-promyelocytic-leukemia-treated-with-arsenic-trioxide-and-all-trans-retinoid-acid
#12
Ursula Creutzig, Michael N Dworzak, Konrad Bochennek, Jörg Faber, Christian Flotho, Norbert Graf, Udo Kontny, Claudia Rossig, Irene Schmid, Arend von Stackelberg, Jans-Enno Mueller, Christine von Neuhoff, Dirk Reinhardt, Nils von Neuhoff
Recently, studies in adults with acute promyelocytic leukemia (APL) showed high cure rates in low-risk patients treated with all-trans retinoid acid (ATRA) and arsenic trioxide (ATO), while toxicities were significantly reduced compared to the standard treatment with ATRA and chemotherapy. Here we report about first experience with 11 pediatric patients with low-risk APL treated with ATRA and ATO. All patients stayed in molecular remission. All suffered from hyperleukocytosis. Two patients experienced reversible severe side effects...
January 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28108543/disease-characteristics-and-prognostic-implications-of-cell-surface-flt3-receptor-cd135-expression-in-pediatric-acute-myeloid-leukemia-a-report-from-the-children-s-oncology-group
#13
Katherine Tarlock, Todd A Alonzo, Michael R Loken, Robert B Gerbing, Rhonda E Ries, Richard Aplenc, Lillian Sung, Susana Raimondi, Betsy A Hirsch, Samir B Kahwash, Amy McKenney, E Anders Kolb, Alan S Gamis, Soheil Meshinchi
PURPOSE: The FLT3 cell-surface receptor tyrosine kinase (CD135) is expressed in a majority of both acute lymphoid leukemia (ALL) and myeloid leukemia (AML). However, the prognostic significance of CD135 expression in AML remains unclear. We therefore evaluated the association between FLT3 surface expression and disease characteristics and outcomes in pediatric patients with AML. EXPERIMENTAL DESIGN: We analyzed FLT3 receptor expression on AML blasts by multi-dimensional flow cytometry and its association with disease characteristics, clinical outcomes, and FLT3 transcript level in 367 children with AML treated on the Children's Oncology Group trial AAML0531...
January 20, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28097792/fish-identifies-a-kat6a-crebbp-fusion-caused-by-a-cryptic-insertional-t-8-16-in-a-case-of-spontaneously-remitting-congenital-acute-myeloid-leukemia-with-a-normal-karyotype
#14
Rachel Barrett, Barbara Morash, David Roback, Chantale Pambrun, Lesley Marfleet, Rhett P Ketterling, Karen Harrison, Jason N Berman
Cytogenetics can inform risk stratification in pediatric acute myeloid leukemia (AML). We describe the first case of a newborn with leukemia cutis found to have AML harboring a cryptic insertional t(8;16)(p11.2;p13.3) with associated KAT6A/CREBBP fusion identified exclusively by fluorescence in situ hybridization (FISH). Expectant management resulted in spontaneous leukemia resolution. The identification of t(8;16)(p11.2;p13.3) may serve as a biomarker for spontaneous remission in congenital AML. FISH for this translocation is warranted in congenital AML with a normal karyotype, and patients with KAT6A/CREBBP fusion should be conservatively managed...
January 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28091414/acute-myeloid-leukemia-advancements-in-diagnosis-and-treatment
#15
REVIEW
Meng-Ge Yu, Hu-Yong Zheng
OBJECTIVE: Leukemia is the most common pediatric malignancy and a major cause of morbidity and mortality in children. Among all subtypes, a lack of consensus exists regarding the diagnosis and treatment of acute myeloid leukemia (AML). Patient survival rates have remained modest for the past three decades in AML. Recently, targeted therapy has emerged as a promising treatment. DATA SOURCES: We searched the PubMed database for recently published research papers on diagnostic development, target therapy, and other novel therapies of AML...
January 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28089099/clinical-characteristics-and-outcome-of-invasive-fungal-infections-in-pediatric-acute-myeloid-leukemia-patients-in-a-medical-center-in-taiwan
#16
Gu-Lung Lin, Hsiu-Hao Chang, Chun-Yi Lu, Chung-Ming Chen, Meng-Yao Lu, Ping-Ing Lee, Shiann-Tarng Jou, Yung-Li Yang, Li-Min Huang, Luan-Yin Chang
BACKGROUND: Invasive fungal infection (IFI) causes significant morbidity and mortality in patients with hematological malignancies, especially those with acute myeloid leukemia (AML), recurrent acute leukemia, high-risk acute lymphoblastic leukemia, and after allogeneic hematopoietic stem cell transplantation. The study aimed to investigate the clinical characteristics and outcome of IFIs in pediatric AML patients in a medical center in Taiwan. METHODS: We performed retrospective chart reviews...
December 19, 2016: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/28067901/targeting-samhd1-with-the-vpx-protein-to-improve-cytarabine-therapy-for-hematological-malignancies
#17
Nikolas Herold, Sean G Rudd, Linda Ljungblad, Kumar Sanjiv, Ida Hed Myrberg, Cynthia B J Paulin, Yaser Heshmati, Anna Hagenkort, Juliane Kutzner, Brent D G Page, José M Calderón-Montaño, Olga Loseva, Ann-Sofie Jemth, Lorenzo Bulli, Hanna Axelsson, Bianca Tesi, Nicholas C K Valerie, Andreas Höglund, Julia Bladh, Elisée Wiita, Mikael Sundin, Michael Uhlin, Georgios Rassidakis, Mats Heyman, Katja Pokrovskaja Tamm, Ulrika Warpman-Berglund, Julian Walfridsson, Sören Lehmann, Dan Grandér, Thomas Lundbäck, Per Kogner, Jan-Inge Henter, Thomas Helleday, Torsten Schaller
The cytostatic deoxycytidine analog cytarabine (ara-C) is the most active agent available against acute myelogenous leukemia (AML). Together with anthracyclines, ara-C forms the backbone of AML treatment for children and adults. In AML, both the cytotoxicity of ara-C in vitro and the clinical response to ara-C therapy are correlated with the ability of AML blasts to accumulate the active metabolite ara-C triphosphate (ara-CTP), which causes DNA damage through perturbation of DNA synthesis. Differences in expression levels of known transporters or metabolic enzymes relevant to ara-C only partially account for patient-specific differential ara-CTP accumulation in AML blasts and response to ara-C treatment...
February 2017: Nature Medicine
https://www.readbyqxmd.com/read/28063963/timing-and-utility-of-relapse-surveillance-after-allogeneic-hematopoietic-cell-transplantation-in-children-with-leukemia
#18
Anya Levinson, Staci Arnold, Zhezhen Jin, Monica Bhatia, Diane George, James H Garvin, Prakash Satwani
The utility and optimal timing of routine bone marrow (BM) and cerebrospinal fluid (CSF) surveillance after allogeneic hematopoietic cell transplantation (alloHCT) in children with leukemia have not been previously studied. To examine the current practice concerning relapse surveillance in this population, we conducted a national survey of pediatric bone marrow transplant physicians. Sixty-two of 152 potential participants (41%) completed the survey. For acute lymphoblastic leukemia (ALL) patients, 41 physicians (66%) reported performing routine BM analysis in all such patients, 15 (24%) in some patients and 6 (10%) in no patients...
January 4, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28063196/asxl2-mutations-are-frequently-found-in-pediatric-aml-patients-with-t-8-21-runx1-runx1t1-and-associated-with-a-better-prognosis
#19
Genki Yamato, Norio Shiba, Kenichi Yoshida, Yuichi Shiraishi, Yusuke Hara, Kentaro Ohki, Jun Okubo, Haruna Okuno, Kenichi Chiba, Hiroko Tanaka, Akitoshi Kinoshita, Hiroshi Moritake, Nobutaka Kiyokawa, Daisuke Tomizawa, Myoung-Ja Park, Manabu Sotomatsu, Takashi Taga, Souichi Adachi, Akio Tawa, Keizo Horibe, Hirokazu Arakawa, Satoru Miyano, Seishi Ogawa, Yasuhide Hayashi
ASXL2 is an epigenetic regulator involved in polycomb repressive complex regulation or recruitment. Clinical features of pediatric acute myeloid leukemia (AML) patients with ASXL2 mutations remain unclear. Thus, we investigated frequencies of ASXL1 and ASXL2 mutations, clinical features of patients with these mutations, correlations of these mutations with other genetic alterations including BCOR/BCORL1 and cohesin complex component genes, and prognostic impact of these mutations in 369 pediatric patients with de novo AML (0-17 years)...
May 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28063190/prognostic-impact-of-specific-molecular-profiles-in-pediatric-acute-megakaryoblastic-leukemia-in-non-down-syndrome
#20
Yusuke Hara, Norio Shiba, Kentaro Ohki, Ken Tabuchi, Genki Yamato, Myoung-Ja Park, Daisuke Tomizawa, Akitoshi Kinoshita, Akira Shimada, Hirokazu Arakawa, Akiko M Saito, Nobutaka Kiyokawa, Akio Tawa, Keizo Horibe, Takashi Taga, Souichi Adachi, Tomohiko Taki, Yasuhide Hayashi
Pediatric acute megakaryoblastic leukemia in non-Down syndrome (AMKL) is a unique subtype of acute myeloid leukemia (AML). Novel CBFA2T3-GLIS2 and NUP98-KDM5A fusions recurrently found in AMKL were recently reported as poor prognostic factors. However, their detailed clinical and molecular characteristics in patients treated with recent improved therapies remain uncertain. We analyzed molecular features of 44 AMKL patients treated on two recent Japanese AML protocols, the AML99 and AML-05 trials. We identified CBFA2T3-GLIS2, NUP98-KDM5A, RBM15-MKL1, and KMT2A rearrangements in 12 (27%), 4 (9%), 2 (5%), and 3 (7%) patients, respectively...
May 2017: Genes, Chromosomes & Cancer
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