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https://www.readbyqxmd.com/read/28804660/large-granular-lymphocytic-leukemia-a-report-of-response-to-rituximab
#1
Uroosa Ibrahim, Sara Parylo, Shiksha Kedia, Shafinaz Hussein, Jean Paul Atallah
Large granular lymphocytic (LGL) leukemia is a rare form of low grade leukemia characterized by large cytotoxic T cells or natural killer cells on morphological examination. Immunosuppressive therapy is employed as first-line therapy. Treatment options in refractory cases include the anti-CD52 antibody alemtuzumab and purine analogues. We report a rare case that responded to the anti-CD20 monoclonal antibody rituximab. A 77-year-old female presented with complaints of fatigue, fever, and chills of 3 months' duration...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28723629/stat3-mutation-impacts-biological-and-clinical-features-of-t-lgl-leukemia
#2
Antonella Teramo, Gregorio Barilà, Giulia Calabretto, Chiara Ercolin, Thierry Lamy, Aline Moignet, Mikael Roussel, Cédric Pastoret, Matteo Leoncin, Cristina Gattazzo, Anna Cabrelle, Elisa Boscaro, Sara Teolato, Elisa Pagnin, Tamara Berno, Elena De March, Monica Facco, Francesco Piazza, Livio Trentin, Gianpietro Semenzato, Renato Zambello
STAT3 mutations have been described in 30-40% of T-large granular lymphocyte (T-LGL) leukemia patients, leading to STAT3 pathway activation. Considering the heterogeneity of the disease and the several immunophenotypes that LGL clone may express, the aim of this work was to evaluate whether STAT3 mutations might be associated with a distinctive LGL immunophenotype and/or might be indicative for specific clinical features.Our series of cases included a pilot cohort of 101 T-LGL leukemia patients (68 CD8+/CD4- and 33 CD4+/CD8±) from Padua Hematology Unit (Italy) and a validation cohort of additional 20 patients from Rennes Hematology Unit (France)...
June 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28717070/clinical-features-pathogenesis-and-treatment-of-large-granular-lymphocyte-leukemias
#3
Kazuo Oshimi
Large granular lymphocytes (LGLs) are large lymphocytes with azurophilic granules in their cytoplasm. LGLs are either natural killer (NK) cells or T lymphocytes. Expansions of the LGLs in the peripheral blood are seen in various conditions, including three clonal disorders: T-cell LGL (T-LGL) leukemia, chronic lymphoproliferative disorders of NK cells (CLPD-NK), and aggressive NK-cell leukemia (ANKL). However, the monoclonal and polyclonal expansion of LGLs has been associated with many other conditions. The present article describes these LGL disorders, with special emphasis on the clinical features, pathogenesis, and treatments of the three above-mentioned clonal disorders...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28701716/glyat-regulates-jnk-mediated-cell-death-in-drosophila
#4
Pu Ren, Wenzhe Li, Lei Xue
Cell death is a fundamental progress that regulates cell number, tissue homeostasis and organ size in development. The c-Jun N-terminal kinase (JNK) pathway has been evolutionarily conserved from fly to human, and plays essential roles in regulating cell death. To characterize additional genes that regulate JNK signaling, we performed a genetic screen in Drosophila and identified dGLYAT, a novel gene whose function was previously unknown, as a modulator of JNK-mediated cell death. We found that loss of dGLYAT suppressed JNK activation and cell death triggered by over-expression of Egr or Hep, or depletion of puc or lgl in development, suggesting dGLYAT regulates both ectopic and physiological functions of JNK pathway...
July 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28674237/study-of-bantam-mirna-expression-in-brain-tumour-resulted-due-to-loss-of-polarity-modules-in-drosophila-melanogaster
#5
Animesh Banerjee, Jagat K Roy
Disturbance of delicate concordance between stem cell proliferation, specification and differentiation during brain development leads to several neural disorders including tumours. Accumulating evidences have demonstrated involvement of short noncoding microRNAs (miRNAs) in governing several biological as well as pathological processes, including tumourigenesis across various species. Drosophila bantam miRNA, known to regulate critical physiological functions is reported to have elevated expression in ovarian tumour...
June 2017: Journal of Genetics
https://www.readbyqxmd.com/read/28611255/a-kinome-rnai-screen-in-drosophila-identifies-novel-genes-interacting-with-lgl-apkc-and-crb-cell-polarity-genes-in-epithelial-tissues
#6
Linda M Parsons, Nicola A Grzeschik, Kasun Amaratunga, Peter Burke, Leonie M Quinn, Helena E Richardson
In both Drosophila melanogaster and mammalian systems, epithelial structure and underlying cell polarity are essential for proper tissue morphogenesis and organ growth. Cell polarity interfaces with multiple cellular processes that are regulated by the phosphorylation status of large protein networks. To gain insight into the molecular mechanisms that coordinate cell polarity with tissue growth, we screened a boutique collection of RNAi stocks targeting the kinome for their capacity to modify Drosophila "cell polarity" eye and wing phenotypes...
August 7, 2017: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/28556532/feline-large-granular-lymphocyte-lymphoma-an-italian-society-of-veterinary-oncology-sioncov-retrospective-study
#7
R Finotello, M E Vasconi, S Sabattini, C Agnoli, C Giacoboni, M Annoni, A Dentini, G Bettini, P Guazzi, D Stefanello, E Bottero, P Mesto, R Marinelli, C De Feo, L Marconato
Feline large granular lymphocyte (LGL) lymphoma is an uncommon subtype of lymphoma characterized by a grave prognosis and scarce response to chemotherapy. There are limited reports on clinico-pathological and prognostic factors. One-hundred and 9 cats with newly diagnosed LGL lymphoma that underwent initial staging (including hematology, serum biochemistry, thoracic radiographs and abdominal ultrasound), and followed-up were retrospectively evaluated. LGL lymphoma was localized within the gastrointestinal tract with or without extra-intestinal involvement in 91...
May 29, 2017: Veterinary and Comparative Oncology
https://www.readbyqxmd.com/read/28552558/llgl1-connects-cell-polarity-with-cell-cell-adhesion-in-embryonic-neural-stem-cells
#8
Yves Jossin, Minhui Lee, Olga Klezovitch, Elif Kon, Alexia Cossard, Wen-Hui Lien, Tania E Fernandez, Jonathan A Cooper, Valera Vasioukhin
Malformations of the cerebral cortex (MCCs) are devastating developmental disorders. We report here that mice with embryonic neural stem-cell-specific deletion of Llgl1 (Nestin-Cre/Llgl1(fl/fl)), a mammalian ortholog of the Drosophila cell polarity gene lgl, exhibit MCCs resembling severe periventricular heterotopia (PH). Immunohistochemical analyses and live cortical imaging of PH formation revealed that disruption of apical junctional complexes (AJCs) was responsible for PH in Nestin-Cre/Llgl1(fl/fl) brains...
June 5, 2017: Developmental Cell
https://www.readbyqxmd.com/read/28454252/multifactorial-neutropenia-in-a-patient-with-acute-promyelocytic-leukemia-and-associated-large-granular-lymphocyte-expansion-a-case-report
#9
Gianluigi Reda, Bruno Fattizzo, Ramona Cassin, Elena Flospergher, Nicola Orofino, Umberto Gianelli, Wilma Barcellini, Agostino Cortelezzi
Neutropenia in the setting of acute hematological malignancies may impact disease prognosis, thus affecting therapy dose intensity. This is often due to chemotherapy-induced aplasia as well as to the disease itself. However, chronic neutropenia deserves further investigation, as the management of reversible concomitant causes may avoid treatment delay. The present study describes a case of an acute promyelocytic leukemia patient with chronic severe neutropenia of multifactorial origin, including acute leukemia itself, chemotherapy, autoimmune activation with anti-platelets and anti-neutrophil antibodies positivity, and the rare association of large granular lymphocyte (LGL) expansion...
March 2017: Oncology Letters
https://www.readbyqxmd.com/read/28449187/distinctive-clinical-characteristics-and-favorable-outcomes-in-patients-with-large-granular-lymphocytosis-after-allo-hct-12-year-follow-up-data
#10
Marc Poch Martell, Nada Hamad, Elizabeth Shin, Joon Ho Moon, Sang Kyun Sohn, Jieun Uhm, Fotios V Michelis, Auro Viswabandya, Jeffrey H Lipton, Hans A Messner, Dennis Dong Hwan Kim
An increase in large granular lymphocytes (LGL) is frequently seen in patients following allogeneic hematopoietic cell transplantation (allo-HCT) and it has been associated with better outcomes in some reports. We assessed 826 consecutive patients at our institution with over 12 years of follow-up for the occurrence of LGL lymphocytosis after allo-HCT. The 3-year cumulative incidence of LGL lymphocytosis was 14.5% with a median duration of over 3.5 years. The development of LGL lymphocytosis was strongly correlated with CMV viremia and GVHD...
April 27, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28427176/metronomic-regimen-as-an-effective-treatment-for-aggressive-t-lgl-leukemia-with-central-nervous-system-infiltration-clinical-experience-and-review-of-literature
#11
REVIEW
Yun Liu, Lei Fan, Huihui Zhao, Wei Xu, Jianyong Li
A 71-year-old man was diagnosed with T-Large granular lymphocytic (LGL) leukemia, which usually represents a relatively indolent clinical course. While the clinical manifestation of this patient we report herein was aggressive with lasting fever, splenomegaly and hemophagocytic lymphohistiocytosis (HLH). T-cell immunophenotype was CD3+CD4-CD8-CD5-CD7-TCRαβ+. After comprehensive evaluation, an adjusted chemotherapy regimen CEOP (cyclophosphamide, vincristine, etoposide, prednisone) with etoposide, a potential effective regimen for HLH was administrated to the patient...
May 9, 2017: Oncotarget
https://www.readbyqxmd.com/read/28407967/molecular-characterization-of-an-unauthorized-genetically-modified-bacillus-subtilis-production-strain-identified-in-a-vitamin-b2-feed-additive
#12
Valentina Paracchini, Mauro Petrillo, Ralf Reiting, Alexandre Angers-Loustau, Daniela Wahler, Andrea Stolz, Birgit Schönig, Anastasia Matthies, Joachim Bendiek, Dominik M Meinel, Sven Pecoraro, Ulrich Busch, Alex Patak, Joachim Kreysa, Lutz Grohmann
Many food and feed additives result from fermentation of genetically modified (GM) microorganisms. For vitamin B2 (riboflavin), GM Bacillus subtilis production strains have been developed and are often used. The presence of neither the GM strain nor its recombinant DNA is allowed for fermentation products placed on the EU market as food or feed additive. A vitamin B2 product (80% feed grade) imported from China was analysed. Viable B. subtilis cells were identified and DNAs of two bacterial isolates (LHL and LGL) were subjected to three whole genome sequencing (WGS) runs with different devices (MiSeq, 454 or HiSeq system)...
September 1, 2017: Food Chemistry
https://www.readbyqxmd.com/read/28407008/dysregulated-signaling-proliferation-and-apoptosis-impact-on-the-pathogenesis-of-tcr%C3%AE-%C3%AE-t-cell-large-granular-lymphocyte-leukemia
#13
Martine J Kallemeijn, Dick de Ridder, Joyce Schilperoord-Vermeulen, Michèle Y van der Klift, Yorick Sandberg, Jacques J M van Dongen, Anton W Langerak
TCRγδ+ T-LGL leukemia is a rare form of chronic mature T cell disorders in elderly, which is generally characterized by a persistently enlarged CD3+CD57+TCRγδ+ large granular lymphocyte population in the peripheral blood with a monoclonal phenotype. Clinically, the disease is heterogeneous, most patients being largely asymptomatic, although neutropenia, fatigue and B symptoms and underlying diseases such as autoimmune diseases or malignancies are also often observed. The etiology of TCRγδ+ T-LGL proliferations is largely unknown...
2017: PloS One
https://www.readbyqxmd.com/read/28401952/correlations-in-the-degeneracy-of-structurally-controllable-topologies-for-networks
#14
Colin Campbell, Steven Aucott, Justin Ruths, Derek Ruths, Katriona Shea, Réka Albert
Many dynamic systems display complex emergent phenomena. By directly controlling a subset of system components (nodes) via external intervention it is possible to indirectly control every other component in the system. When the system is linear or can be approximated sufficiently well by a linear model, methods exist to identify the number and connectivity of a minimum set of external inputs (constituting a so-called minimal control topology, or MCT). In general, many MCTs exist for a given network; here we characterize a broad ensemble of empirical networks in terms of the fraction of nodes and edges that are always, sometimes, or never a part of an MCT...
April 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28161176/association-among-gestation-length-and-health-production-and-reproduction-in-holstein-cows-and-implications-for-their-offspring
#15
A Vieira-Neto, K N Galvão, W W Thatcher, J E P Santos
Objectives were to evaluate associations among gestation length (GL) and performance in Holstein cows and their offspring. A total of 8,095 Holstein cows and 3,635 female offspring born alive on 2 farms using only artificial insemination (AI) were evaluated. Gestation length averaged 276 ± 6 d in the 8,095 dams, and it was categorized as short (SGL; at least 1 SD below the population mean; mean = 266 d, range 256 to 269 d), average (AGL; population mean ± 1 SD; mean = 276 d, range 270 to 282 d), or long (LGL; at least 1 SD above the population mean; mean = 285 d, range 283 to 296 d)...
April 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/28134920/lymphocyte-expansion-after-unrelated-cord-blood-allogeneic-stem-cell-transplantation-in-adults
#16
Y Le Bris, T Guillaume, A Ménard, M Illiaquer, J Martin, S Malard, A Duquesne, P Peterlin, C Debord, N Robillard, M Eveillard, S Wuillème, J Delaunay, M Mohty, A Garnier, P Moreau, M C Béné, P Chevallier
Limited information is available regarding the incidence and features of lymphocyte expansions after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Large granular lymphocytes (LGL) expansions have been reported after bone marrow or peripheral blood, but not after unrelated cord blood (UCB) allo-HSCT, associated with indolent clinical courses and favorable outcomes. Here, we considered 85 recipients of UCB allo-HSCT to more broadly define the impact of lymphocytosis, not limited to LGL. Sustained lymphocytosis was observed in 21 (25%) patients at a median onset of 12...
June 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28128670/large-granular-lymphocytic-leukemia-current-diagnostic-and-therapeutic-approaches-and-novel-treatment-options
#17
REVIEW
Estella Matutes
Large granular lymphocytic leukemia (LGLL) is a low grade lymphoproliferative disorder characterized by the clonal proliferation of large granular lymphocytes (LGL) and recognised by the WHO. The diagnosis and management of these patients is challenging due to the limited information from prospective studies. Guidelines for front-line therapy have not been established. The prognosis is favourable with median overall survivals greater than 10 years. Areas covered: This manuscript is a review of the clinical features, diagnosis, pathogenesis and, in particular, the various available therapeutic options for this rare lymphoid leukemia...
March 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28115367/lgl-leukemia-from-pathogenesis-to-treatment
#18
REVIEW
Thierry Lamy, Aline Moignet, Thomas P Loughran
Large granular lymphocyte (LGL) leukemia has been recognized by the World Health Organization classifications amongst mature T-cell and natural killer (NK) cell neoplasms. There are 3 categories: chronic T-cell leukemia and NK-cell lymphocytosis, which are similarly indolent diseases characterized by cytopenias and autoimmune conditions as opposed to aggressive NK-cell LGL leukemia. Clonal LGL expansion arise from chronic antigenic stimulation, which promotes dysregulation of apoptosis, mainly due to constitutive activation of survival pathways including Jak/Stat, MapK, phosphatidylinositol 3-kinase-Akt, Ras-Raf-1, MEK1/extracellular signal-regulated kinase, sphingolipid, and nuclear factor-κB...
March 2, 2017: Blood
https://www.readbyqxmd.com/read/28048675/tu-fg-209-07-medical-physics-1-0-versus-medical-physics-2-0-a-case-study
#19
D Carver, C Willis, P Stauduhar, T Nishino, J Wells, E Samei
PURPOSE: To illustrate how performance analytics can identify performance decrement in digital radiography systems. METHODS: Subsequent to a radiologist's image quality complaint, four different advanced methods contributed to root cause analysis. Our system was a GE Revolution XQi digital radiography unit. Initially, we reviewed weekly GE Quality Assurance Procedures (QAP) results in a database dating from 2001. Next, we evaluated objective image quality metrics of individual PA Chest radiographs acquired...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/27957496/evaluation-of-lethal-giant-larvae-as-a-schistosomiasis-vaccine-candidate
#20
Yufan Cao, Hongbin Qiao, Yanli Shi, Yu Han, Jinming Liu, Hao Li, Ke Lu, Jiaojiao Lin, Yamei Jin
Schistosomiasis is a neglected tropical disease of humans, and it is considered to be the second most devastating parasitic disease after malaria. Eggs produced by normally developed female worms are important in the transmission of the parasite, and they responsible for the pathogenesis of schistosomiasis. The tumor suppressor gene lethal giant larvae (lgl) has an essential function in establishing apical-basal cell polarity, cell proliferation, differentiation, and tissue organization. In our earlier study, downregulation of the lgl gene induced a significant reduction in the egg hatching rate of Schistosoma japonicum (Sj) eggs...
2016: BioMed Research International
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