keyword

chronic neutrophilic leukemia

keyword
https://read.qxmd.com/read/31697825/inherited-transmission-of-the-csf3r-t618i-mutational-hotspot-in-familial-chronic-neutrophilic-leukemia
#1
Nicolas Duployez, Christophe Willekens, Isabelle Plo, Alice Marceau-Renaut, Stéphane de Botton, Laurène Fenwarth, Thomas Boyer, Guillemette Huet, Olivier Nibourel, Christian Rose, Brigitte Nelken, Bruno Quesnel, Claude Preudhomme
No abstract text is available yet for this article.
October 31, 2019: Blood
https://read.qxmd.com/read/31697804/jak2ex13indel-drives-oncogenic-transformation-and-is-associated-with-chronic-eosinophilic-leukemia-and-polycythemia-vera
#2
Ami B Patel, Anca Franzini, Emilie Leroy, Soo Jin Kim, Anthony D Pomicter, Lidvine Genet, Michael Xiao, Dongqing Yan, Jonathan Ahmann, Archana M Agarwal, Phillip Clair, Juanah Addada, Jonathan Lambert, Matthew Salmon, Gerald J Gleich, Nicholas Cp Cross, Stefan Constantinescu, Thomas O'Hare, Josef T Prchal, Michael W Deininger
The V617F mutation in the JH2 domain of JAK2 is an oncogenic driver in several myeloproliferative neoplasms (MPNs), including essential thrombocythemia, myelofibrosis, and polycythemia vera (PV). Other mutations in JAK2 have been identified in MPNs, most notably exon 12 mutations in PV. Here, we describe a novel recurrent mutation characterized by a common 4-amino acid deletion and variable 1-amino acid insertion (Leu583-Ala586DelInsSer/Gln/Pro) within the JH2 domain of JAK2. All four affected patients had eosinophilia, and both patients with Leu583-Ala586DelInsSer fulfilled diagnostic criteria of both PV and chronic eosinophilic leukemia (CEL)...
October 11, 2019: Blood
https://read.qxmd.com/read/31695542/polycythemia-vera-associated-complications-pathogenesis-clinical-manifestations-and-effects-on-outcomes
#3
REVIEW
Danielle Cuthbert, Brady Lee Stein
Polycythemia vera is a Philadelphia-negative chronic myeloproliferative neoplasm, characterized by erythrocytosis, which is unique, compared to essential thrombocytosis and primary myelofibrosis. Though longevity can usually be expected, vascular morbidity is associated with this condition, as well as a propensity to evolve into myelofibrosis (post-PV MF) and acute myeloid leukemia. In addition, patients can have a pronounced symptom burden. Herein, contributors to the symptomatic burden, as well as the thrombotic and transformative tendencies are reviewed...
2019: Journal of Blood Medicine
https://read.qxmd.com/read/31692115/next-generation-sequencing-reveals-unique-combination-of-mutations-in-cis-of-csf3r-in-atypical-chronic-myeloid-leukemia
#4
Jae Won Yun, Jung Yoon, Chul Won Jung, Ki-O Lee, Jong Won Kim, Sun-Hee Kim, Hee-Jin Kim
BACKGROUND: Atypical chronic myeloid leukemia (aCML) is a hematologic disorder characterized by leukocytosis with increased dysplastic neutrophils and their precursors. In CSF3R gene, the activation mutation including T618I is frequently reported in aCML but is rarely accompanied by truncation mutations. Herein, we report a unique aCML patient with two CSF3R mutations (T618I and Y779*) in the same DNA strand. METHODS: High-coverage next-generation sequencing for 40 genes related with myeloid leukemia was performed...
November 6, 2019: Journal of Clinical Laboratory Analysis
https://read.qxmd.com/read/31657787/beclin-1-as-a-neutrophil-specific-immune-checkpoint
#5
Yu-Lin Su, Marcin Kortylewski
Neutrophils are early wound healing and inflammation regulators that, due to functional plasticity, can adopt either pro- or antitumor functions. Until recently, beclin-1 was a protein known mainly for its role as a critical regulator of autophagy. In this issue of the JCI, Tan et al. describe the effects of the beclin-1 conditional myeloid cell-specific deletion in mice, in which immunostimulation resulted in hypersensitive neutrophils. The chronic proinflammatory effect of these neutrophils triggered spontaneous B cell malignancies to develop...
October 28, 2019: Journal of Clinical Investigation
https://read.qxmd.com/read/31655606/dasatinib-regulates-lps-induced-microglial-and-astrocytic-neuroinflammatory-responses-by-inhibiting-akt-stat3-signaling
#6
Ka-Young Ryu, Hyun-Ju Lee, Hanwoong Woo, Ri-Jin Kang, Kyung-Min Han, HyunHee Park, Sang Min Lee, Ju-Young Lee, Yoo Joo Jeong, Hyun-Wook Nam, Youngpyo Nam, Hyang-Sook Hoe
BACKGROUND: The FDA-approved small-molecule drug dasatinib is currently used as a treatment for chronic myeloid leukemia (CML). However, the effects of dasatinib on microglial and/or astrocytic neuroinflammatory responses and its mechanism of action have not been studied in detail. METHODS: BV2 microglial cells, primary astrocytes, or primary microglial cells were treated with dasatinib (100 or 250 nM) or vehicle (1% DMSO) for 30 min or 2 h followed by lipopolysaccharide (LPS; 200 ng/ml or 1 μg/ml) or PBS for 5...
October 26, 2019: Journal of Neuroinflammation
https://read.qxmd.com/read/31650185/nitric-oxide-and-peroxynitrite-trigger-and-enhance-release-of-neutrophil-extracellular-traps
#7
Aneta Manda-Handzlik, Weronika Bystrzycka, Adrianna Cieloch, Eliza Glodkowska-Mrowka, Ewa Jankowska-Steifer, Edyta Heropolitanska-Pliszka, Agnieszka Skrobot, Angelika Muchowicz, Olga Ciepiela, Malgorzata Wachowska, Urszula Demkow
Despite great interest, the mechanism of neutrophil extracellular traps (NETs) release is not fully understood and some aspects of this process, e.g. the role of reactive nitrogen species (RNS), still remain unclear. Therefore, our aim was to investigate the mechanisms underlying RNS-induced formation of NETs and contribution of RNS to NETs release triggered by various physiological and synthetic stimuli. The involvement of RNS in NETs formation was studied in primary human neutrophils and differentiated human promyelocytic leukemia cells (HL-60 cells)...
October 24, 2019: Cellular and Molecular Life Sciences: CMLS
https://read.qxmd.com/read/31648329/safety-of-allogeneic-hematopoietic-cell-transplant-in-adults-after-cd19-targeted-car-t-cell-therapy
#8
Mazyar Shadman, Jordan Gauthier, Kevin A Hay, Jenna M Voutsinas, Filippo Milano, Ang Li, Alexandre V Hirayama, Mohamed L Sorror, Sindhu Cherian, Xueyan Chen, Ryan D Cassaday, Brian G Till, Ajay K Gopal, Brenda M Sandmaier, David G Maloney, Cameron J Turtle
Allogeneic hematopoietic cell transplantation (allo-HCT) is offered to selected patients after chimeric antigen receptor-modified T-cell (CAR-T) therapy. Lymphodepleting chemotherapy and CAR-T therapy have immunosuppressive and immunomodulatory effects that could alter the safety profile of subsequent allo-HCT. We reviewed our experience with 32 adults (acute lymphoblastic leukemia [ALL], n = 19; B-cell non-Hodgkin lymphoma [NHL]/chronic lymphocytic leukemia [CLL], n = 13) who received an allo-HCT after CAR-T therapy, with a focus on posttransplant toxicities...
October 22, 2019: Blood Advances
https://read.qxmd.com/read/31639635/ibrutinib-based-therapy-impaired-neutrophils-microbicidal-activity-in-patients-with-chronic-lymphocytic-leukemia-during-the-early-phases-of-treatment
#9
Alessandro Prezzo, Filomena Monica Cavaliere, Caterina Bilotta, Tancredi Massimo Pentimalli, Metello Iacobini, Laura Cesini, Robin Foà, Francesca Romana Mauro, Isabella Quinti
Ibrutinib is a tyrosine kinase inhibitor used in the treatment of a variety of lymphoid malignancies, including chronic lymphocytic leukemia (CLL). Drugs inhibiting B-cell-receptor (BCR)-associated kinases, including BTK inhibitors, act on B cells and on a wide spectrum of tissues and cells, including innate immunity cells. Thus, alterations in the Bruton's tyrosine kinase (BTK) kinase function could lead to an impairment of innate immune cells functions and to an increased infectious risk in patients receiving BTK inhibitors...
October 3, 2019: Leukemia Research
https://read.qxmd.com/read/31597841/novel-treatment-strategies-for-myeloproliferative-neoplasms
#10
Prithviraj Bose, Lucia Masarova, Srdan Verstovsek
Although the Janus kinase (JAK) inhibitor ruxolitinib has long been the only drug licensed for treatment of the classic Philadelphia chromosome negative (Ph- ) myeloproliferative neoplasms, years of drug development efforts have begun to bear fruit with the recent approval of a novel monopegylated interferon alfa-2b, ropeginterferon alfa, for patients with polycythemia vera without symptomatic splenomegaly in Europe. Several newer JAK inhibitors (fedratinib, pacritinib, momelotinib) have shown activity in phase 3 trials in patients with myelofibrosis but have, for various reasons, not yet received regulatory approval; all these agents, however, remain in active clinical development...
2019: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://read.qxmd.com/read/31546004/prospective-phase-2-study-of-umbilical-cord-blood-transplantation-in-adult-acute-leukemia-and-myelodysplastic-syndrome
#11
Seitaro Terakura, Tetsuya Nishida, Masashi Sawa, Tomonori Kato, Kotaro Miyao, Yukiyasu Ozawa, Akio Kohno, Yasushi Onishi, Noriko Fukuhara, Masanobu Kasai, Nobuharu Fujii, Hisayuki Yokoyama, Hiroatsu Iida, Nobuhiro Kanemura, Atsushi Fujieda, Hiroatsu Ago, Yutaka Tsutsumi, Fumihiko Nakamura, Kazuhiro Yago, Yukiyoshi Moriuchi, Shuichi Ota, Haruhiko Ohashi, Atsumi Yanagisawa, Ritsuro Suzuki, Yachiyo Kuwatsuka, Yoshiko Atsuta, Koichi Miyamura, Makoto Murata
Almost comparable transplant outcomes were reported between HLA-matched unrelated donor transplantation (UDT) and cord blood transplantation (CBT). Herein, we conducted a prospective phase 2 study to assess efficacy and safety of single-unit myeloablative CBT in adult leukemia and myelodysplastic syndrome. Because day 180 survival of UDT was approximately 80%, we determined the alternative hypothesis of expected day 180 survival with successful engraftment rate as 80% and set the null hypothesis of threshold rate as 65%...
September 20, 2019: Biology of Blood and Marrow Transplantation
https://read.qxmd.com/read/31484888/-rapidly-fatal-streptococcal-toxic-shock-syndrome-due-to-streptococcus-agalactiae-in-a-patient-with-chronic-myeloid-leukemia-and-cirrhosis
#12
Hiroko Iizuka, Yasutaka Fukuda, Yosuke Mori, Noriaki Iwao, Michiaki Koike, Masaaki Noguchi, Norio Komatsu
A 72-year-old woman with chronic myeloid leukemia (CML) and cirrhosis complicated with blood blisters on her right upper arm and ascites was admitted. She presented with shock vital on admission. Initial gram staining of blood cultures showed gram-positive cocci in chains, suggesting streptococcal toxic shock syndrome (STSS). Amputation of the right upper arm was performed owing to necrotizing fasciitis. Despite continued antibiotic therapy and systemic management, the blood blisters rapidly spread to the skin of the whole body, and she died 41 h after admission...
2019: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://read.qxmd.com/read/31418388/-efficacy-analysis-of-unrelated-cord-blood-transplantation-for-high-risk-refractory-aml1-eto-positive-myeloid-leukemia
#13
Jia-Rong Ren, Xiao-Yu Zhu, Bao-Lin Tang, Xiang Wan, Juan Tong, Lei Zhang, Xu-Han Zhang, Kai-Di Song, Wen Yao, Guang-Yu Sun, Hui-Lan Liu, Zi-Min Sun
OBJECTIVE: To analyze the clinical outcomes of engraftment, graft-versus-host disease (GVHD) and survival in the patients with AML1-ETO positive acute myeloid leukemia (AML) treated with unrelated umbilical cord blood transplantation (UCBT). METHODS: Forty-Five patients with high-risk refractory AML1-ETO positive AML were treated with a single UCBT in a single center from July 2010 to April 2018. All the patients underwent a myeloablative preconditioning regimen,and cyclosporine A (CSA) combined with mycophenolate mofetil (MMF) was used to prevent GVHD...
August 2019: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://read.qxmd.com/read/31408646/paraneoplastic-hyperleukocytosis-mimicking-hematologic-malignancy-revealing-a-localized-lung-cancer
#14
Amira Marouf, Nicolas Chapuis, Anne Sophie Alary, Barbara Burroni, Olivier Kosmider, Marco Alifano, Didier Bouscary
Paraneoplastic leukemoid reaction is a challenging differential diagnosis when it presents at the time of diagnosis of cancer. Severe hyperleukocytosis with elevation of blood neutrophils and monocytes counts can evoke myeloid hematologic malignancies. We report the case of a patient who presented with blood and bone marrow features highly suggestive of chronic myelomonocytic leukemia. The diagnosis of primary lung sarcomatoid carcinoma was performed. Surgical removal of this tumor which will always remain the priority led to full normalization of blood cell count...
August 10, 2019: Annals of Thoracic Surgery
https://read.qxmd.com/read/31377458/targeted-sequencing-aids-in-identifying-clonality-in-chronic-myelomonocytic-leukemia
#15
Sang Mee Hwang, Sung-Min Kim, Youngwon Nam, Jinhyun Kim, Sungsik Kim, Yong-Oon Ahn, Yong Park, Sung-Soo Yoon, Sue Shin, Sunghoon Kwon, Dong Soon Lee
Chronic myelomonocytic leukemia (CMML) typically shows monocytosis in the peripheral blood (PB), which must be differentiated from reactive monocytosis. To determine the clonality of CMML, we performed molecular and cytogenetic analysis in Korean patients. To investigate whether monocytes in the PB harbored clonal mutational changes, we performed single-cell sequencing after selecting monocytes, neutrophils, and lymphocytes by morphology-aided laser microdissection. Targeted sequencing was performed in 35 patients with CMML with 41 bone marrow samples...
September 2019: Leukemia Research
https://read.qxmd.com/read/31366621/genomic-landscape-of-neutrophilic-leukemias-of-ambiguous-diagnosis
#16
Haijiao Zhang, Beth Wilmot, Daniel Bottomly, Kim-Hien T Dao, Emily Stevens, Christopher A Eide, Vishesh Khanna, Angela Rofelty, Samantha Savage, Anna Reister Schultz, Nicola Long, Libbey White, Amy Carlos, Rachel Henson, Chenwei Lin, Robert Searles, Robert H Collins, Daniel J DeAngelo, Michael W Deininger, Tamara Dunn, Than Hein, Marlise R Luskin, Bruno C Medeiros, Stephen T Oh, Daniel A Pollyea, David P Steensma, Richard M Stone, Brian J Druker, Shannon K McWeeney, Julia E Maxson, Jason R Gotlib, Jeffrey W Tyner
Chronic neutrophilic leukemia (CNL), atypical chronic myeloid leukemia (aCML), and myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN-U) are a group of rare and heterogeneous myeloid disorders. There is strong morphologic resemblance among these distinct diagnostic entities as well as a lack of specific molecular markers and limited understanding of disease pathogenesis, which has made diagnosis challenging in certain cases. The treatment has remained empirical, resulting in dismal outcomes...
September 12, 2019: Blood
https://read.qxmd.com/read/31340617/-hla-10-10-matched-unrelated-donor-versus-sibling-donor-hematopoietic-stem-cell-transplantation-for-adult-acute-myeloid-leukemia
#17
J F Yao, G X Zhang, Y Y Shen, R L Zhang, Y He, J L Wei, E L Jiang, D L Yang, S Z Feng, M Z Han
Objective: To evaluate the outcomes of human leukocyte antigen (HLA) matched unrelated donor hematopoietic stem cell transplantation (MUD-HSCT) for adult acute myeloid leukemia (AML) in a single center. Methods: Consecutive adult AML who received MUD-HSCT in our center from January 2008 to April 2017 were studied retrospectively, comparing with patients undergoing matched sibling donor (MSD) -HSCT in the same period. The rates of overall survival (OS) , disease free survival (DFS) , relapse, non-relapse mortality (NRM) , engraftment, acute and chronic graft-versus-host disease (aGVHD and cGVHD) were analyzed...
June 14, 2019: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://read.qxmd.com/read/31312310/-a-no-scarring-persistent-skin-lesion-for-3-years-pyoderma-gangrenosum
#18
Michel Assane Ndour, Djiby Sow, Demba Diedhiou, Fabrice Senghor, Muriel Diembou, Moustapha Diouf, Awa Faye, Ibrahima Mané Diallo, Ahmed Limane Barrage, Léa Marie Kabou, Maimouna Ndour Mbaye, Anna Sarr
Pyoderma gangrenosum (PG) is a rare non-infectious neutrophilic dermatosis often unknowed. It usually presents with inflammatory skin ulcer, very painful, with rapid evolution. It is commonly found in a context of malignancy, inflammatory bowel disease, rheumatic and/or haematological disease. Its diagnosis is very often late after multiple therapeutic failures. We report a case of pyoderma gangrenosum whose diagnosis was not obvious. A patient was admitted to our department for a persistent dermatological lesion and adverse evolution despite debridements and the administration of antibiotics...
2019: Pan African Medical Journal
https://read.qxmd.com/read/31308356/a-case-of-papillary-thyroid-carcinoma-and-kostmann-syndrome-a-genomic-theranostic-approach-for-comprehensive-treatment
#19
Soo Han, John Ehrhardt, Savya Shukla, Adel Elkbuli, Yuri E Nikiforov, Seza A Gulec
BACKGROUND Theranostics is a combined diagnostic and treatment approach to individualized patient care. Kostmann syndrome, or severe congenital neutropenia, is an autosomal recessive disease that affects the production of neutrophils. Papillary thyroid carcinoma (PTC) is the most common type of thyroid malignancy associated with gene alterations, including in the mitogen-activated protein kinase (MAPK) signaling pathway gene. Translocation of the ETS variant 6/neurotrophic receptor tyrosine kinase 3 (ETV6/NTRK3) gene has been implicated in radiation-induced and pediatric forms of thyroid carcinoma but has rarely been described in sporadic PTC...
July 16, 2019: American Journal of Case Reports
https://read.qxmd.com/read/31306778/successful-outcome-in-patients-with-fanconi-anemia-undergoing-t-cell-replete-mismatched-related-donor-hematopoietic-cell-transplantation-using-reduced-dose-cyclophosphamide-post-transplantation
#20
Mouhab Ayas, Khawar Siddiqui, Abdullah Al-Jefri, Ali Al-Ahmari, Ibrahim Ghemlas, Hawazen Al-Saedi, Awatif Alanazi, Rafat Jafri, Mohamad F Ayas, Amal Al-Seraihi
Allogeneic hematopoietic cell transplantation (HCT) has been shown to restore normal hematopoiesis in patients with Fanconi anemia (FA), with excellent results in matched related donor HCT. Outcomes of alternative donor HCT are less favorable, however. In patients without FA, several reports have documented stable engraftment and/or a low risk of graft-versus-host disease (GVHD) using unmanipulated HLA-mismatched related donors and post-HCT cyclophosphamide (PT-CY) for GVHD prophylaxis. Data on the use of this approach in patients with FA are scarce, and thus we launched a study of HLA-mismatched related donor HCT in these patient...
July 12, 2019: Biology of Blood and Marrow Transplantation
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