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https://www.readbyqxmd.com/read/30501706/-clinical-charcteristics-and-prognostic-analysis-of-28-cases-of-pediatric-myelodysplastic-syndrome
#1
Jian Wang, Shao-Fen Lin, Qi-Hui Chen, Kun-Yin Qiu, Hong-Gui Xu, Ke Huang, Yang Li, Jian-Pei Fang, Zhou Dun-Hua Zhou
OBJECTIVE: To analyze the clinical features and prognosis of 28 children with myelodysplastic syndrome (MDS) and to screen the high risk factors affecting the prognosis so as to provide the new ideas for standard of clinical diagnosis and therapy. METHODS: The clinical data of 28 children with newly diagnosed MDS treated in our hospital from March 1994 to July 2016 were analyzed retrospectively, the features of disease onset and the results of laboratory examination were summarized, all MDS children were followed up, the prognosis and the high risk factors affecting the prognosis were evaluated...
December 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/30450160/knock-in-of-the-wt1-r394w-mutation-causes-mds-and-cooperates-with-flt3-itd-to-drive-aggressive-myeloid-neoplasms-in-mice
#2
Colleen E Annesley, Cara Rabik, Amy S Duffield, Rachel E Rau, Daniel Magoon, Li Li, Vicki Huff, Donald Small, David M Loeb, Patrick Brown
Wilms tumor 1 (WT1) is a zinc finger transcriptional regulator, and has been implicated as both a tumor suppressor and oncogene in various malignancies. Mutations in the DNA-binding domain of the WT1 gene are described in 10-15% of normal-karyotype AML (NK-AML) in pediatric and adult patients. Similar WT1 mutations have been reported in adult patients with myelodysplastic syndrome (MDS). WT1 mutations have been independently associated with treatment failure and poor prognosis in NK-AML. Internal tandem duplication (ITD) mutations of FMS-like tyrosine kinase 3 ( FLT3 ) commonly co-occur with WT1 -mutant AML, suggesting a cooperative role in leukemogenesis...
October 19, 2018: Oncotarget
https://www.readbyqxmd.com/read/30334483/-change-in-paradigm-in-the-treatment-of-pediatric-acquired-bone-marrow-failure-syndromes-in-hungary
#3
Krisztián Kállay, Judit Csomor, Emma Ádám, Csaba Bödör, Csaba Kassa, Réka Simon, Gábor Kovács, György Péter, Gábor Ottóffy, Katalin Bartyik, Csongor Kiss, Péter Masát, Marienn Réti, Blanka Tóth, Gergely Kriván
INTRODUCTION: Acquired bone marrow failures are rare but fatal diseases in childhood. Since 2013, Hungary has been participating as a full member in the work of the European Working Group on uniform diagnostics and therapy in patients with acquired bone marrow failure syndromes. Hypocellular refractory cytopenia of childhood has been emphasized as a frequent entity, transplanted by reduced intensity conditioning with excellent outcomes. AIM: To analyse and compare the results of treatment before and after our joining...
October 2018: Orvosi Hetilap
https://www.readbyqxmd.com/read/30305537/-progress-in-research-of-the-pathogenesis-of-childhood-mds-mpn
#4
Kenichi Yoshida
Recent research reveals novel insights into the pathogenesis of childhood myelodysplastic syndromes (MDS) in addition to that of juvenile myelomonocytic leukemia (JMML). In pediatric MDS, the genetic characteristics of which have been barely elucidated previously, germline mutations, particularly those in GATA2, SAMD9, and SAML9L, have been frequently identified, indicating the importance of germline predisposition in childhood MDS compared with adult MDS. In JMML, in addition to the known Ras-pathway mutations, novel secondary mutations and causative fusion genes have been reported...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/30303870/acute-megakaryoblastic-leukemia-with-diffuse-periosteal-reaction-of-bilateral-lower-extremities
#5
Erin E Doherty, Erica K Schallert, Alexandra M Stevens, Jyotinder N Punia, Jennifer H Foster
We report the case of a 3-year-old girl diagnosed with acute megakaryoblastic leukemia, who presented after >1 year of bilateral leg pain. At times the pain was severe enough to prevent ambulation, prompting visits to her primary care provider. However, it was not until acute respiratory failure occurred with subsequent hospitalization in the pediatric intensive care unit that severe anemia and thrombocytopenia were discovered and the diagnosis of acute myeloid leukemia was made. Bilateral lower extremity swelling was noted on admission and radiographs showed diffusely abnormal appearance of the long bones of her lower extremities with periosteal reaction and echogenic debris in the subperiosteal space, thought to represent leukemic cells...
October 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30088135/long-term-pediatric-hematological-morbidity-of-the-early-term-newborn
#6
Gil Gutvirtz, Tamar Wainstock, Eyal Sheiner, Daniella Landau, Alon Slutzky, Asnat Walfisch
Children born at early term (37 0/7 to 38 6/7 weeks' gestation) are at an increased risk for long-term respiratory, cardiovascular, neurological, and developmental morbidities as compared with children born at full term (39 0/7 to 40 6/7 weeks' gestation). In this population-based cohort analysis, we sought to evaluate the long-term hematological morbidity of early-term born children. The cohort consisted of 223,242 term singleton deliveries. Hospitalizations of the offspring up to 18 years of age involving hematological morbidity were evaluated, including hereditary and acquired anemias, immunodeficiency disorders, coagulation disorders, white blood cell disorders, cytopenias, polycythemia, and myelodysplastic syndrome...
November 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/30047423/monosomy-7-in-pediatric-myelodysplastic-syndromes
#7
REVIEW
Marcin W Wlodarski, Sushree S Sahoo, Charlotte M Niemeyer
Myelodysplastic syndromes (MDS) in children and adolescents are a rare heterogeneous group of clonal stem cell disorders. Complete or partial loss of chromosome 7 constitutes the most common cytogenetic abnormality encountered in any type of childhood MDS, is associated with more advanced disease, and usually requires a timely allogeneic stem cell transplantation. This article provides insights into the current understanding of the genotype, phenotype, and clonal evolution patterns in pediatric MDS associated with loss of chromosome 7...
August 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/30020493/radiation-exposure-from-pediatric-ct-scans-and-subsequent-cancer-risk-in-the-netherlands
#8
Johanna M Meulepas, Cécile M Ronckers, Anne M J B Smets, Rutger A J Nievelstein, Patrycja Gradowska, Choonsik Lee, Andreas Jahnen, Marcel van Straten, Marie-Claire Y de Wit, Bernard Zonnenberg, Willemijn M Klein, Johannes H Merks, Otto Visser, Flora E van Leeuwen, Michael Hauptmann
Background: Computed tomography (CT), a strong diagnostic tool, delivers higher radiation doses than most imaging modalities. As CT use has increased rapidly, radiation protection is important, particularly among children. We evaluate leukemia and brain tumor risk following exposure to low-dose ionizing radiation from CT scans in childhood. Methods: For a nationwide retrospective cohort of 168 394 children who received one or more CT scans in a Dutch hospital between 1979 and 2012 who were younger than age 18 years, we obtained cancer incidence, vital status, and confounder information by record linkage with external registries...
July 18, 2018: Journal of the National Cancer Institute
https://www.readbyqxmd.com/read/30008805/an-uncommon-t-9-11-p24-q22-with-monoallelic-loss-of-atm-and-kmt2a-genes-in-a-child-with-myelodysplastic-syndrome-acute-myeloid-leukemia-who-evolved-from-fanconi-anemia
#9
Viviane Lamim Lovatel, Daiane Corrêa de Souza, Tatiana Fonseca Alvarenga, Roberto R Capela de Matos, Claudia Diniz, Marcia Trindade Schramm, Juan Clinton Llerena Júnior, Maria Luiza Macedo Silva, Eliana Abdelhay, Teresa de Souza Fernandez
Background: Myelodysplastic syndrome (MDS) is rare in the pediatric age group and it may be associated with inheritable bone marrow failure (BMF) such as Fanconi anemia (FA). FA is a rare multi-system genetic disorder, characterized by congenital malformations and progressive BMF. Patients with FA usually present chromosomal aberrations when evolving to MDS or acute myeloid leukemia (AML). Thus, the cytogenetic studies in the bone marrow (BM) of these patients have an important role in the therapeutic decision, mainly in the indication for hematopoietic stem cell transplantation (HSCT)...
2018: Molecular Cytogenetics
https://www.readbyqxmd.com/read/29891567/clonal-dynamics-of-donor-derived-myelodysplastic-syndrome-after-unrelated-hematopoietic-cell-transplantation-for-high-risk-pediatric-b-lymphoblastic-leukemia
#10
Jason R Schwartz, Michael P Walsh, Jing Ma, Tamara Lamprecht, Shuoguo Wang, Gang Wu, Susana Raimondi, Brandon M Triplett, Jeffery M Klco
Donor-derived hematologic malignancies are rare complications of hematopoietic cell transplantation (HCT). Although these are commonly either a myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML), in general, they are a heterogeneous group of diseases, and a unified mechanism for their development has remained elusive. Here we report next-generation sequencing, including whole-exome sequencing (WES), whole-genome sequencing (WGS), and targeted sequencing, of a case of donor-derived MDS (dMDS) following HCT for high-risk B-lymphoblastic leukemia (B-ALL) in an adolescent...
October 2018: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29888021/sacral-myeloid-sarcoma-manifesting-as-radiculopathy-in-a-pediatric-patient-an-unusual-form-of-myeloid-leukemia-relapse
#11
Joana Ruivo Rodrigues, Manuel João Brito, Rui Pedro Faria Pais, Sílvia Carvalho
Myeloid sarcoma (MS), granulocytic sarcoma or chloroma, is defined as a localized extramedullary mass of blasts of granulocytic lineage with or without maturation, occurring outside the bone marrow. MS can be diagnosed concurrently with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). The authors report a case of sacral MS occurring as a relapse of myeloid leukemia in a 5-year-old girl who was taken to the emergency department with radiculopathy symptoms.
2018: Case Reports in Radiology
https://www.readbyqxmd.com/read/29803285/acquired-aplastic-anemia-what-have-we-learned-and-what-is-in-the-horizon
#12
REVIEW
Süreyya Savaşan
Acquired aplastic anemia (aAA) characterized by peripheral pancytopenia and bone marrow aplasia is a rare and serious disorder. Differential diagnosis includes constitutional bone marrow failure syndromes and myelodysplastic disorders. Autoimmune reaction to altered hematopoietic stem cells highlights the underlying mechanism. Matched related donor allogeneic hematopoietic stem cell transplantation is the ideal pediatric treatment; alternative approaches include immunosuppressive therapy and use of eltrombopag...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29753157/treosulfan-fludarabine-and-low-dose-total-body-irradiation-for-children-and-young-adults-with-acute-myeloid-leukemia-or-myelodysplastic-syndrome-undergoing-allogeneic-hematopoietic-cell-transplantation-prospective-phase-ii-trial-of-the-pediatric-blood-and-marrow
#13
Eneida R Nemecek, Ralf A Hilger, Alexia Adams, Bronwen E Shaw, Deidre Kiefer, Jennifer Le-Rademacher, John E Levine, Gregory Yanik, Wing Leung, Julie-An Talano, Paul Haut, David Delgado, Neena Kapoor, Aleksandra Petrovic, Roberta Adams, Rabi Hanna, Hemalatha Rangarajan, Jignesh Dalal, Joseph Chewning, Michael R Verneris, Stacy Epstein, Lauri Burroughs, Evelio D Perez-Albuerne, Michael A Pulsipher, Colleen Delaney
This multicenter study evaluated a treosulfan-based regimen in children and young adults with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS) undergoing allogeneic hematopoietic cell transplant (HCT). Forty patients with median age 11 years (range, 1 to 19) underwent allogeneic HCT for AML in first (n = 18), second (n = 11), and third or greater remission (n = 3) or MDS (n = 8) using bone marrow (n = 25), peripheral blood stem cells (n = 5), or cord blood (n = 9). The regimen consisted of body surface area (BSA)-based treosulfan 10 g/m2 /day (BSA ≤ ...
August 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29742616/spontaneous-remission-of-monosomy-7-six-years-after-diagnosis
#14
Holly J Edington, Eric J Lowe
Monosomy 7 may be a poor prognostic indicator in pediatric myelodysplastic syndrome. There are case reports of children with monosomy 7 who undergo spontaneous remission 2 to 24 months after diagnosis. We report a case of a child with bone marrow failure and monosomy 7 who underwent spontaneous remission 75 months after diagnosis. The patient had no exposure to chemotherapeutic or immunosuppressive agents. The patient did not receive chemotherapy or other treatment during the 75 months. Despite remaining positive for monosomy 7, he never developed myelodysplasia or acute myeloid leukemia...
May 8, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683949/a-rare-case-of-hemophagocytic-lymphohistiocytosis-associated-with-myelodysplastic-syndrome-and-trisomy-8-in-a-pediatric-patient
#15
Seh Hyun Kim, Dae Yong Yi, Na Mi Lee, Sin Weon Yun, Soo Ahn Chae, In Seok Lim
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting in clinical and biochemical manifestations of extreme inflammation. Myelodysplastic syndrome (MDS) represents a heterogenous group of clonal hematopoietic disorders. The development of MDS is common in children with trisomy of chromosome 8. Here, we report a fatal case of 8-year-old girl who was admitted to the emergency department with status epilepticus, and later diagnosed with HLH associated with MDS and trisomy of chromosome 8. We believe this is the first reported case of HLH associated with MDS and trisomy 8 in a pediatric patient...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29667765/molecular-profile-of-inflammatory-and-megakaryocytic-factors-in-pediatric-myelodysplastic-syndrome-with-acute-myelofibrosis
#16
Kais Hussein, Meinolf Suttorp, Angelika Stucki-Koch, Irith Baumann, Charlotte M Niemeyer, Hans Kreipe
Pediatric fibrotic myelodysplastic syndromes (ped-MDS-MF) and pediatric primary myelofibrosis (ped-PMF) are rare, and the molecular changes which mediate fibrosis have never been investigated. Histology and gene expression profile of 119 fibrosis/angiogenesis/inflammation/megakaryopoiesis-related factors in bone marrow biopsies were performed (two ped-MDS-MF and one ped-PMF). In one progressive ped-MDS, comparison of MF grade 0 (no myelofibrosis) and MF grade 2 (dense network of reticulin fibres) after 4 months showed that expression of fibrosis-related transcripts increased and dysplastic megakaryocytes formed a dense net of CD42b+ proplatelets...
July 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29659673/evaluation-of-the-utility-of-bone-marrow-morphology-and-ancillary-studies-in-pediatric-patients-under-surveillance-for-myelodysplastic-syndrome
#17
Karen M Chisholm, Min Xu, Billy Davis, Amy Ogi, M Cristina Pacheco, Amy E Geddis, Karen D Tsuchiya, Joe C Rutledge
Objectives: To evaluate the utility of flow cytometry, karyotype, and a fluorescence in situ hybridization (FISH) panel in screening children for myelodysplastic syndrome (MDS). Methods: Bone marrow morphology, flow cytometry, karyotype, and FISH reports from 595 bone marrow specimens (246 patients) were analyzed. Results: By morphology, 8.7% of cases demonstrated at least unilineage dysplasia and/or increased blasts. Flow cytometry identified definitive abnormalities in 2...
April 25, 2018: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29608607/pharmacokinetics-adapted-busulfan-based-myeloablative-conditioning-before-unrelated-umbilical-cord-blood-transplantation-for-myeloid-malignancies-in-children
#18
Joy Benadiba, Marc Ansari, Maja Krajinovic, Marie-France Vachon, Michel Duval, Pierre Teira, Sonia Cellot, Henrique Bittencourt
Unrelated umbilical cord blood transplantation (UCBT) is an alternative to provide transplants in children with acute leukemia or myelodysplastic syndrome who lack a related donor. Intravenous Busulfan (Bu) combined with therapeutic drug monitoring-guided dosing has been increasingly used, with more predictable bioavailability and better outcomes comparing to oral Bu. There is still an important variation in Bu pharmacokinetic between patients that is associated with an increased risk of toxicity and graft failure...
2018: PloS One
https://www.readbyqxmd.com/read/29509314/the-use-of-antimicrobial-photodynamic-therapy-in-oral-injuries-of-a-pediatric-patient-with-myelodysplastic-syndrome-case-report
#19
Maria Cecília Freire de Melo, Thuanny Silva de Macedo, Julianelly Alves Biserra, Karina Luz Cavalcanti Rodrigues, Mônica Cruz, Aurora Karla de Lacerda Vidal
AIM: To report a case of a pediatric patient carrier of myelodysplastic syndrome (MDS) with severe oral infectious disease, in which antimicrobial photodynamic therapy (aPDT) was used as a therapeutic choice to support systemic treatment. METHODS AND RESULTS: This case report refers to a 1-year-old male patient with MDS and hospitalized for investigation and treatment of complications at a Pediatric Oncology and Hematology Center in Recife/PE, Brazil. The intraoral examination revealed a violaceous lesion surrounding the lower incisor teeth, which progressed with alteration of color to milky yellowish white, compromising the entire lower gingival border, leading to tooth mobility and consequent loss of teeth 71 and 81...
March 2018: Special Care in Dentistry
https://www.readbyqxmd.com/read/29481579/loss-of-full-length-gata1-expression-in-megakaryocytes-is-a-sensitive-and-specific-immunohistochemical-marker-for-the-diagnosis-of-myeloid-proliferative-disorder-related-to-down-syndrome
#20
Winston Y Lee, Olga K Weinberg, Andrew G Evans, Geraldine S Pinkus
Objectives: Myeloid proliferative disorders associated with Down syndrome (MPD-DS), including transient abnormal myelopoiesis and myeloid leukemia associated with Down syndrome (DS), harbor mutations of GATA1, a transcription factor essential for erythroid and megakaryocytic development. These mutations result in a N-terminally truncated GATA1 (GATA1s) and prohibit the production of the full-length GATA1 (GATA1f). Here, we demonstrate the utility of immunohistochemical GATA1f reactivity in diagnosing MPD-DS...
March 7, 2018: American Journal of Clinical Pathology
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