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https://www.readbyqxmd.com/read/29143068/patient-characteristics-and-outcomes-in-adolescents-and-young-adults-with-classical-philadelphia-chromosome-negative-myeloproliferative-neoplasms
#1
Prajwal Boddu, Lucia Masarova, Srdan Verstovsek, Paolo Strati, Hagop Kantarjian, Jorge Cortes, Zeev Estrov, Sherry Pierce, Naveen Pemmaraju
Little is known about the outcomes of Philadelphia-negative myeloproliferative neoplasms (MPNs) in adolescents and young adults (AYA). We reviewed all patients with essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF) treated at our institution from 1988 to 2016 who were aged 16 to 39 years (AYA) and described their outcomes in comparison to older MPN population. Of 2206 patients, 185 (8.3%) were identified as AYA: 105 (57%) ET, 43 (23%) PV, and 37 (20%) MF. The median age was 33 years [range, 16-39], and median follow-up time 3 years [range, 0...
November 15, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29140408/philadelphia-chromosome-like-mixed-phenotype-acute-leukemia-demonstrating-p2ry8-crlf2-fusion-and-jak1-mutation
#2
Sarah M Choi, John K Frederiksen, Charles W Ross, Dale L Bixby, Lina Shao
Objectives: Philadelphia chromosome-like (Ph-like) genetic alterations define a subset of B lymphoblastic leukemia/lymphoma (B-ALL), which represents a separate provisional entity in the World Health Organization 2016 updated classification. However, these alterations have not been described outside the context of B-ALL. Methods: Cytogenomic array and molecular analysis identified a Ph-like signature in a mixed-phenotype acute leukemia (MPAL), B/myeloid, confirmed using conventional immunophenotypic and cytochemical analysis...
November 11, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29133777/acute-lymphoblastic-leukemia-patient-with-variant-atf7ip-pdgfrb-fusion-and-favorable-response-to-tyrosine-kinase-inhibitor-treatment-a-case-report
#3
Ge Zhang, Yanle Zhang, Jianrong Wu, Yan Chen, Zhigui Ma
BACKGROUND Chromosomal translocations involving the PDGFRB gene have been reported in a broad spectrum of hematological malignancies. An ATF7IP/PDGFRB fusion was recently identified in a Philadelphia chromosome-like (Ph-like) B-progenitor acute lymphoblastic leukemia (B-ALL) patient. Here we report on a special case of a Ph-like ALL patient who had a variant ATF7IP/PDGFRB fusion. CASE REPORT In this case, a variant fusion was created between ATF7IP exon 9 (instead of exon 13) and PDGFRB exon 11, resulting in the loss of 411 nucleotides and 137 amino acids in the ATF7IP/PDGFRB fusion cDNA and its encoded chimeric protein, respectively...
November 14, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29132472/-research-progress-in-ph-like-childhood-acute-lymphoblastic-leukemia
#4
Xue Tang, Xia Guo
Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL) is a subtype of B-lineage ALL (B-ALL) that displays a gene expression profile (GEP) similar to Philadelphia chromosome-positive ALL (Ph(+) ALL). It has a diverse range of genetic alterations that activate cytokine receptor genes and kinase signaling pathways, frequently accompanied by abnormal transcription factors related to lymphatic development. Children with Ph-like ALL account for 15% of children with high-risk B-ALL. It has adverse clinical features and a poor prognosis...
November 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29121435/altered-intracellular-signaling-by-imatinib-increases-the-anti-cancer-effects-of-tyrosine-kinase-inhibitors-in-cml-cells
#5
Takuya Hirao, Masashi Yamaguchi, Megumi Kikuya, Hiroji Chibana, Kousei Ito, Shigeki Aoki
Tyrosine kinase inhibitors (TKIs), including imatinib (IM), improve the outcome of chronic myelogenous leukemia (CML) therapy. However, TKI treatment is long-term and can induce resistance to TKIs, which often leads to a poor clinical outcome in CML patients. Here, we examined the effect of continuous IM exposure on intracellular energy metabolism in K562 cells, a human Philadelphia chromosome-positive CML cell line, and its subsequent sensitivity to anti-cancer agents. Contrary to our expectations, we found that continuous IM exposure increased sensitivity to TKIs...
November 9, 2017: Cancer Science
https://www.readbyqxmd.com/read/29118670/an-overview-of-the-role-of-platelets-in-angiogenesis-apoptosis-and-autophagy-in-chronic-myeloid-leukaemia
#6
REVIEW
Lisa Repsold, Roger Pool, Mohammed Karodia, Gregory Tintinger, Annie Margaretha Joubert
Amongst males, leukaemia is the most common cause of cancer-related death in individuals younger than 40 years of age whereas in female children and adolescents, leukaemia is the most common cause of cancer-related death. Chronic myeloid leukaemia (CML) is a chronic leukaemia of the haematopoietic stem cells affecting mostly adults. The disease results from a translocation of the Philadelphia chromosome in stem cells of the bone marrow. CML patients usually present with mild to moderate anaemia and with decreased, normal, or increased platelet counts...
2017: Cancer Cell International
https://www.readbyqxmd.com/read/29113859/treatment-decision-for-occipital-arteriovenous-malformations-avms-to-achieve-hemorrhagic-control-while-maximizing-visual-preservation-our-experience-and-review-of-literature
#7
REVIEW
Wuyang Yang, Jose L Porras, Eunice Philadelphia, Jody Law, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander L Coon, Rafael J Tamargo, Judy Huang
Despite concern of hemorrhagic risk, patients with occipital AVMs are at significant risk for visual disturbances after treatment. We aim to characterize the hemorrhage risk and visual disturbance in occipital AVMs patients from our experience and literature review. We performed retrospective review of occipital AVM patients seen at our institution from 1990 to 2015. Patient characteristics were compared using multivariable logistic regression with follow-up visual disturbance as the outcome. We also systematically reviewed the PubMed database for English literature describing occipital AVMs (with exclusion of case reports)...
November 4, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29112080/%C3%AE-lactam-therapeutic-drug-management-in-the-picu
#8
Jeffrey J Cies, Wayne S Moore, Adela Enache, Arun Chopra
OBJECTIVES: To determine whether contemporary β-lactam anti-infective dosing recommendations in critically ill children achieve concentrations associated with maximal anti-infective activity. The secondary objective was to describe the microbiological and clinical outcomes associated with β-lactam therapeutic drug management. DESIGN: Electronic Medical Record Review. SETTING: A 189-bed, freestanding children's tertiary care teaching hospital in Philadelphia, PA...
November 3, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/29103074/diagnostic-accuracy-of-synovial-fluid-blood-markers-and-microbiological-testing-in-chronic-knee-prosthetic-infections
#9
Giovanni Balato, Vincenzo Franceschini, Tiziana Ascione, Alfredo Lamberti, Fiamma Balboni, Andrea Baldini
INTRODUCTION: This retrospective study was undertaken to define cut-off values for synovial fluid (SF) leukocyte count and neutrophil percentage for differentiating aseptic failure and periprosthetic joint infection (PJI) and to evaluate the diagnostic accuracy of blood inflammatory markers, and microbiological testing according to the criteria proposed by the International Consensus Meeting (ICM) of Philadelphia. METHODS: All patients who underwent revision total knee arthroplasty from January 2010 to July 2015 were included: we identified and classified 31 PJIs and 136 aseptic joints...
November 4, 2017: Archives of Orthopaedic and Trauma Surgery
https://www.readbyqxmd.com/read/29093755/unique-amplification-of-bcr-abl1-gene-fusion-in-a-case-of-t-cell-acute-lymphoblastic-leukemia
#10
Rima Koka, Najeebah A Bade, Edward A Sausville, Yi Ning, Ying Zou
Background: ABL1 gene translocations can be seen in precursor T-acute lymphoblastic leukemia (T-ALL). The typical translocation partner is the NUP214 gene. BCR-ABL translocations are relatively rare in this entity. Furthermore, while there have been unique patterns of amplification noted among the NUP214-ABL fusion genes, there have been few such reports among cases with BCR-ABL fusion genes. Case presentation: Here we report a unique case of a 44-year old patient with T-ALL in which the blasts demonstrated a derivative chromosome 9 involving a 9;22 translocation and a dicentric Philadelphia chromosome 22 with a homogeneously staining region at the interface of the 9;22 translocation, leading to BCR-ABL1 gene amplification...
2017: Molecular Cytogenetics
https://www.readbyqxmd.com/read/29091557/single-dose-gene-replacement-therapy-for-spinal-muscular-atrophy
#11
Jerry R Mendell, Samiah Al-Zaidy, Richard Shell, W Dave Arnold, Louise R Rodino-Klapac, Thomas W Prior, Linda Lowes, Lindsay Alfano, Katherine Berry, Kathleen Church, John T Kissel, Sukumar Nagendran, James L'Italien, Douglas M Sproule, Courtney Wells, Jessica A Cardenas, Marjet D Heitzer, Allan Kaspar, Sarah Corcoran, Lyndsey Braun, Shibi Likhite, Carlos Miranda, Kathrin Meyer, K D Foust, Arthur H M Burghes, Brian K Kaspar
BACKGROUND: Spinal muscular atrophy type 1 (SMA1) is a progressive, monogenic motor neuron disease with an onset during infancy that results in failure to achieve motor milestones and in death or the need for mechanical ventilation by 2 years of age. We studied functional replacement of the mutated gene encoding survival motor neuron 1 (SMN1) in this disease. METHODS: Fifteen patients with SMA1 received a single dose of intravenous adeno-associated virus serotype 9 carrying SMN complementary DNA encoding the missing SMN protein...
November 2, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29082835/efficacy-and-safety-of-bispecific-t-cell-engager-blinatumomab-and-the-potential-to-improve-leukemia-free-survival-in-b-cell-acute-lymphoblastic-leukemia
#12
Josep-Maria Ribera
Immunotherapy is a promising modality of treatment of neoplastic diseases, including acute lymphoblastic leukemia (ALL). The CD19/CD3-bispecific T cell-engaging (BiTE®) monoclonal antibody blinatumomab can transiently bind cytotoxic T cells to CD19(+) target B cells of ALL inducing their serial lysis. Areas covered: This review focuses on the efficacy and safety of blinatumomab used for the treatment of relapsed/refractory (R/R) ALL and minimal residual disease (MRD)-positive B-cell precursor (BCP) ALL in adults and children, as well as the future prospects of this drug in the treatment of ALL...
October 30, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/29080398/interdisciplinary-skills-review-program-to-improve-team-responses-during-postpartum-hemorrhage
#13
Marianne Bittle, Kathleen O'Rourke, Sindhu K Srinivas
OBJECTIVE: To develop an interdisciplinary, interactive, skills review program to improve team responses during a postpartum hemorrhage (PPH). DESIGN: Online didactic modules in combination with an interdisciplinary skills program consisting of seven hemorrhage-related stations. SETTING/LOCAL PROBLEM: The project was conducted in the Women's Health Department in a quaternary-care Magnet- and Baby Friendly-designated academic medical center in Philadelphia, Pennsylvania...
October 25, 2017: Journal of Obstetric, Gynecologic, and Neonatal Nursing: JOGNN
https://www.readbyqxmd.com/read/29076138/current-challenges-and-opportunities-in-treating-adult-patients-with-philadelphia-negative-acute-lymphoblastic-leukaemia
#14
REVIEW
Ofir Wolach, Irina Amitai, Daniel J DeAngelo
Significant advances have been made in recent years in the field of Philadelphia-negative acute lymphoblastic leukaemia (ALL). New insights into the biology and genetics of ALL as well as novel clinical observations and new drugs are changing the way we diagnose, risk-stratify and treat adult patients with ALL. New genetic subtypes and alterations refine risk stratification and uncover new actionable therapeutic targets. The incorporation of more intensive, paediatric and paediatric-inspired approaches for young adults seem to have a positive impact on survival in this population...
October 26, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29075538/an-unsuspected-finding-of-t-9-22-a-rare-case-of-philadelphia-chromosome-positive-b-lymphoblastic-lymphoma
#15
Prajwal Boddu, C Cameron Yin, Rashmi Kanagal-Shamanna, Guillin Tang, Beenu Thakral, Tapan Kadia, Marina Konopleva, Elias Jabbour, Nitin Jain
While rare, cases of isolated extramedullary disease of B-cell Lymphoblastic Lymphoma (B-LBL) without morphologic bone marrow involvement have been described. In this report, we illustrate the case of an elderly gentleman who presented with isolated testicular and vertebral LBL involvement but had no morphologic bone marrow involvement. The initial plan of treatment was to treat along the lines of Philadelphia negative B-ALL/LBL. During this time, fluorescence in situ hybridization (FISH) and PCR testing for BCR-ABL1 rearrangements were being performed on the marrow specimens as a part of routine diagnostic workup...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29070998/an-uncommon-presentation-of-a-metachronous-testicular-primary-nonseminoma-and-seminoma-separated-by-two-decades-and-a-testicular-cancer-literature-review
#16
Dennis Andrew Buck, Tristan Dean Smith, Wilbur Nelson Montana
INTRODUCTION: Testicular cancer is the most common malignancy in men aged 15-40 years [Bols et al.: Philadelphia, Wolters Kluwer, Lippincott Williams & Wilkins, 2011]. Its incidence comprises 0.8% of all male cancers worldwide, with a mortality rate of 0.1%. The incidence has nearly doubled from 1975 to 2007 leading to the concern of environmental causes [Thomas: Am J Epidemiol 2013; 178: 1240-1245]. Testicular cancer presents as a painless testicular mass without transillumination. Testicular cancer is subcategorized under germ cell testicular cancer or sex cord-stromal tumors...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29059263/effect-of-financial-incentives-on-glucose-monitoring-adherence-and-glycemic-control-among-adolescents-and-young-adults-with-type-1-diabetes-a-randomized-clinical-trial
#17
Charlene A Wong, Victoria A Miller, Kathryn Murphy, Dylan Small, Carol A Ford, Steven M Willi, Jordyn Feingold, Alexander Morris, Yoonhee P Ha, Jingsan Zhu, Wenli Wang, Mitesh S Patel
Importance: Glycemic control often deteriorates during adolescence and the transition to young adulthood for patients with type 1 diabetes. The inability to manage type 1 diabetes effectively during these years is associated with poor glycemic control and complications from diabetes in adult life. Objective: To determine the effect of daily financial incentives on glucose monitoring adherence and glycemic control in adolescents and young adults with type 1 diabetes...
October 23, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/29058969/public-health-and-the-pope-s-visit-to-philadelphia-2015
#18
James W Buehler, Jessica Caum, Steven J Alles
Pope Francis visited Philadelphia for 2 days during September 2015. Preparedness functions managed by the Philadelphia Department of Public Health (PDPH) were similar to those of other mass gatherings but also required accommodation of special security arrangements and the location of public events in central areas of the city. Public health planning involved collaborations with multiple city, state, and federal agencies and neighboring jurisdictions. PDPH preparations encompassed incident command procedures, contingency planning, disease surveillance and prevention, food safety, vector control, BioWatch air sampling, volunteer management for first-aid services, and continuity of operations...
September 2017: Health Security
https://www.readbyqxmd.com/read/29058281/decitabine-for-relapsed-acute-lymphoblastic-leukemia-after-allogeneic-hematopoietic-stem-cell-transplantation
#19
Jie-Ke Cui, Yin Xiao, Yong You, Wei Shi, Qing Li, Yi Luo, Lin Jiang, Zhao-Dong Zhong
Relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains a main question on treatment failure. Current strategies for management that usually include salvage chemotherapy, donor lymphocytic infusion and second transplantation. Our study assessed the efficacy of decitabine (DAC) for treating patients with acute lymphoblastic leukemia (ALL) who relapsed after allogeneic hematopoietic stem cell transplantation (allo-HSCT). We retrospectively analyzed the outcomes of 12 patients with relapsed ALL after allo-HSCT who received DAC therapy...
October 2017: Journal of Huazhong University of Science and Technology. Medical Sciences
https://www.readbyqxmd.com/read/29052026/high-dose-chemotherapy-and-autologous-peripheral-blood-stem-cell-transplantation-with-bcvac-regimen-followed-by-maintenance-chemotherapy-for-children-with-very-high-risk-acute-lymphoblastic-leukemia
#20
Che Ry Hong, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin, Hyo Seop Ahn
Allogeneic hematopoietic stem cell transplantation (HSCT) is the recommended treatment for children with very high risk acute lymphoblastic leukemia (ALL), but it requires adequate institutional infrastructure, experience, and expertise, especially for alternative donor HSCT. We review our experience with high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (APBSCT), followed by post-APBSCT maintenance chemotherapy for children with very high risk ALL. Between August 1997 and November 2012, our institute was not successful with HLA-haploidentical HSCT...
October 20, 2017: International Journal of Hematology
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