keyword
https://read.qxmd.com/read/38610985/large-granular-lymphocytic-leukemia-clinical-features-molecular-pathogenesis-diagnosis-and-treatment
#1
REVIEW
Fauzia Ullah, Mariam Markouli, Mark Orland, Olisaemeka Ogbue, Danai Dima, Najiullah Omar, Moaath K Mustafa Ali
Large granular lymphocytic (LGL) leukemia is a lymphoproliferative disorder characterized by persistent clonal expansion of mature T- or natural killer cells in the blood via chronic antigenic stimulation. LGL leukemia is associated with specific immunophenotypic and molecular features, particularly STAT3 and STAT5 mutations and activation of the JAK-STAT3 , Fas/Fas-L and NF-κB signaling pathways. Disease-related deaths are mainly due to recurrent infections linked to severe neutropenia. The current treatment is based on immunosuppressive therapies, which frequently produce unsatisfactory long-term responses, and for this reason, personalized approaches and targeted therapies are needed...
March 27, 2024: Cancers
https://read.qxmd.com/read/38562340/sepsis-unveils-t-cell-large-granular-lymphocytic-leukemia-in-the-setting-of-end-stage-renal-disease-a-rare-hematologic-malignancy
#2
Tutul Chowdhury, Kalendra Kunwar, Fareeza Mustafa, Annmarie T Sajeev, Mrinal Sharma, Muhammad N Pasha, Madhumati Kalavar
Large granular lymphocytic (LGL) leukemia is a rare chronic lymphoproliferative disorder originating from natural killer cells or T lymphocytes. In this report, we present the case of a 66-year-old female initially treated for sepsis, with   methicillin-sensitive Staphylococcus aureus  identified on initial blood culture prompting intravenous (IV) antibiotic therapy. The patient met systemic inflammatory response syndrome criteria upon admission due to severe neutropenia. Persistent fever led to neurological symptoms, and imaging revealed lung abnormalities along with chronic changes on the CT scan of the head...
March 2024: Curēus
https://read.qxmd.com/read/38498036/a-lineage-specific-stat5bn642h-mouse-model-to-study-nk-cell-leukemia
#3
JOURNAL ARTICLE
Klara Klein, Sebastian Kollmann, Angela Hiesinger, Julia List, Jonatan Kendler, Thorsten Klampfl, Mehak Randhawa, Jana Trifinopoulos, Barbara Maurer, Reinhard Grausenburger, Christof A Bertram, Richard H Moriggl, Thomas Rülicke, Charles G Mullighan, Agnieszka Witalisz-Siepracka, Wencke Walter, Gregor Hoermann, Veronika Sexl, Dagmar Gotthardt
Patients with T- and NK-cell neoplasms frequently have somatic STAT5B gain-of-function mutations. The most frequent STAT5B mutation is STAT5BN642H, which is known to drive murine T-cell leukemia although its role in NK-cell malignancies is unclear. Introduction of the STAT5BN642H mutation into human NK-cell lines enhances their potential to induce leukemia in mice. We have generated a mouse model that enables tissue-specific expression of STAT5BN642H and have selectively expressed the mutated STAT5B in hematopoietic cells (N642Hvav/+) or exclusively in NK cells (N642HNK/NK)...
March 18, 2024: Blood
https://read.qxmd.com/read/38494286/paraneoplastic-myopathies
#4
REVIEW
Andrew L Mammen
This chapter reviews the association between cancer and the idiopathic inflammatory myopathies (IIM), which includes dermatomyositis (DM), antisynthetase syndrome (ASyS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). Accumulating evidence shows that the risk of a coexisting malignancy is high in patients with DM, especially among those with anti-Tif1γ autoantibodies. Patients with IMNM and no defined autoantibodies also have an increased risk of malignancy. Recent evidence demonstrates that many IBM patients have increased numbers of circulating CD57+ CD8+ T cells, consistent with a diagnosis of large granular lymphocytic leukemia...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38435176/t-large-granular-lymphocytic-leukemia-a-rare-diagnosis-in-a-young-woman-with-fever-necrotic-skin-lesions-and-cytopenias
#5
Mário Ferreira, Joana Paulo, Paulo Ramos, Carolina Padrão, Zélia Neves
T-large granular lymphocytic leukemia (T-LGLL) is a rare lymphoproliferative disorder. The diagnosis is established by identifying an abnormally high number of clonal granular T lymphocytes in the peripheral blood and eventually in the bone marrow, in cases with medullary infiltration. The majority of patients present with symptoms related to neutropenia and this condition may be associated with autoimmune diseases in up to a third of cases. The authors describe the case of a 26-year-old patient admitted with subacute high fever and bullous dermatitis with necrotic lesions with central bullae...
February 2024: Curēus
https://read.qxmd.com/read/38344220/modular-control-of-biological-networks
#6
David Murrugarra, Alan Veliz-Cuba, Elena Dimitrova, Claus Kadelka, Matthew Wheeler, Reinhard Laubenbacher
The concept of control is central to understanding and applications of biological network models. Some of their key structural features relate to control functions, through gene regulation, signaling, or metabolic mechanisms, and computational models need to encode these. Applications of models often focus on model-based control, such as in biomedicine or metabolic engineering. This paper presents an approach to model-based control that exploits two common features of biological networks, namely their modular structure and canalizing features of their regulatory mechanisms...
January 23, 2024: ArXiv
https://read.qxmd.com/read/38268478/hepatosplenic-t-cell-lymphoma-displays-an-original-oyster-shell-cytological-pattern-and-a-distinct-genomic-profile-from-that-of-gamma-delta-t-cell-large-granular-lymphocytic-leukemia
#7
JOURNAL ARTICLE
Anne Desmares, Simon Bouzy, Florian Thonier, Julien Goustille, Francisco Llamas-Gutierrez, Franck Genevieve, Laurane Cottin, Lucile Baseggio, Pierre Lemaire, Carinne Lecoq Lafon, Pascale Cornillet-Lefebvre, Anne-Cécile Galoisy, Chantal Brouzes, Emmanuelle Rault, Elodie Dindinaud, Carole Fleury, Florence Blanc-Jouvan, Soraya Wuilleme, Valérie Bardet, Thierry Fest, Thierry Lamy, Mikael Roussel, Mélanie Pannetier, Cédric Pastoret
Not available.
January 25, 2024: Haematologica
https://read.qxmd.com/read/38252421/klrg1-cell-depletion-as-a-novel-therapeutic-strategy-in-patients-with-mature-t-cell-lymphoma-subtypes
#8
JOURNAL ARTICLE
Bimarzhan Assatova, Robert Willim, Christopher Trevisani, Garrett Haskett, Khyati Maulik Kariya, Kusha Chopra, Sung Rye Park, Michael Yevgeniy Tolstorukov, Sean M McCabe, Jessica Duffy, Abner Louissaint, Jani Huuhtanen, Dipabarna Bhattacharya, Satu Mustjoki, Min Jung Koh, Foster Powers, Elizabeth A Morgan, Lei Yang, Brandy Pinckney, Matthew J Cotton, Andrew Crabbe, Jessica Beth Ziemba, Ian Brain, Tayla B Heavican-Foral, Javeed Iqbal, Ronald Nemec, Anna Baird Rider, Josie Germain Ford, Min Ji Koh, Nora Scanlan, David J Feith, Thomas P Loughran, Won Seog Kim, Jaehyuk Choi, Juliette Roels, Lena Boehme, Tom Putteman, Tom Taghon, Jeffrey A Barnes, P Connor Johnson, Eric D Jacobsen, Steven A Greenberg, David M Weinstock, Salvia Jain
PURPOSE: Develop a novel therapeutic strategy for patients with subtypes of mature T-cell and NK-cell neoplasms. EXPERIMENTAL DESIGN: Primary specimens, cell lines, patient-derived xenograft models, commercially available and proprietary anti-KLRG1 antibodies were used for screening, target, and functional validation. RESULTS: Here we demonstrate that surface KLRG1 is highly expressed on tumor cells in subsets of patients with extranodal NK/T-cell lymphoma (ENKTCL), T-prolymphocytic leukemia (T-PLL) and gamma/delta T-cell lymphoma (G/D TCL)...
January 22, 2024: Clinical Cancer Research
https://read.qxmd.com/read/38238319/the-constitutive-activation-of-stat3-gene-and-its-mutations-are-at-the-crossroad-between-lgl-leukemia-and-autoimmune-disorders
#9
REVIEW
Gianpietro Semenzato, Giulia Calabretto, Antonella Teramo, Vanessa Rebecca Gasparini, Elisa Rampazzo, Gregorio Barilà, Renato Zambello
Type T Large Granular Lymphocyte Leukemia (T-LGLL) is a chronic disorder characterized by the abnormal proliferation of clonal cytotoxic T cells. The intriguing association of T-LGLL with autoimmune and inflammatory diseases, the most prominent example being rheumatoid arthritis, raises questions about the underlying pathophysiologic relationships between these disorders which share several biological and clinical features, most notably neutropenia, which is considered as a clinical hallmark. Recent progress in molecular genetics has contributed to a better understanding of pathogenetic mechanisms, thus moving our knowledge in the field of LGL leukemias forward...
January 18, 2024: Blood Cancer Journal
https://read.qxmd.com/read/38105745/the-complex-relationship-between-large-granular-lymphocyte-leukemia-and-rheumatic-disease
#10
REVIEW
Tony Marchand, Thierry Lamy
INTRODUCTION: Large granular lymphocytic (LGL) leukemia is a rare lymphoproliferative disorder characterized by an expansion of clonal T or NK lymphocytes. Neutropenia-related infections represent the main clinical manifestation. Even if the disease follows an indolent course, most patients will ultimately need treatment in their lifetime. Interestingly, LGL leukemia is characterized by a high frequency of autoimmune disorders with rheumatoid arthritis being the most frequent. AREAS COVERED: This review covers the pathophysiology, clinic-biological features and the advances made in the treatment of LGL leukemia...
December 18, 2023: Expert Review of Clinical Immunology
https://read.qxmd.com/read/38062223/clinical-characteristics-and-treatment-outcomes-of-asian-patients-with-t-cell-large-granular-lymphocytic-leukemia-a-single-center-analysis-of-67-cases
#11
JOURNAL ARTICLE
Taekeun Park, Ja Min Byun, Dong-Yeop Shin, Youngil Koh, Junshik Hong, Sung-Soo Yoon, Yoon Hwan Chang, Inho Kim
Large granular lymphocytic (LGL) leukemia is a clonal lymphoproliferative disorder of LGLs derived from cytotoxic T lymphocytes or natural killer cells. However, the clinical features and treatment responses are still not fully understood because of the rarity of the disease. To describe and assess a cohort of patients with T-cell large granular lymphocytic leukemia (T-LGLL). Single-center, retrospective, observational study. We retrospectively collected the clinical data of patients diagnosed with T-LGLL at Seoul National University Hospital since 2006...
December 8, 2023: Annals of Hematology
https://read.qxmd.com/read/37900492/the-use-of-abatacept-for-the-treatment-of-felty-syndrome-in-rheumatoid-arthritis
#12
Rishika V Chin, Sheila Serin, Ahmed Khan, Keneisha Smith, Suresh Kumar
Felty syndrome is characterized by a triad of rheumatoid arthritis, neutropenia, and splenomegaly that typically occurs in patients with seropositive rheumatoid arthritis. We report a case of an 81-year-old man with a 22-year history of rheumatoid arthritis who developed pancytopenia and neutropenia for one month. An abdominal ultrasound revealed splenomegaly. A bone marrow biopsy excluded large granular lymphocytic leukemia, thus Felty Syndrome was diagnosed. He was treated with a maximum tolerated dose of methotrexate (15 mg once a week) for two months with no improvement in absolute neutrophil count...
September 2023: Curēus
https://read.qxmd.com/read/37873793/transient-leukemoid-reaction-from-t-cell-large-granular-lymphocytes-post-autologous-stem-cell-transplant-in-a-patient-affected-by-hodgkin-lymphoma
#13
Andrea Duminuco, Marina Parisi, Giulio Antonio Milone, Alessandra Cupri, Salvatore Leotta, Giuseppe A Palumbo, Nunziatina Laura Parrinello, Grazia Scuderi, Anna Triolo, Giuseppe Milone
Monoclonal T-cell lymphocytosis has been reported in patients with concomitant autoimmune diseases, viral infections, or immunodeficiencies. Referred to as T-cell large granular lymphocytic leukemia (T-LGLL), most cases cannot identify the triggering cause. Only small case series have been reported in the literature, and no treatment consensus exists. T-cell lymphocytosis may also appear after the transplant of hematopoietic stem cells or solid organs. Rare cases have been reported in patients undergoing autologous stem cell transplant (ASCT) for hematological diseases (including multiple myeloma or non-Hodgkin's lymphoma)...
October 11, 2023: Hematology Reports
https://read.qxmd.com/read/37803848/-chidamide-treatment-for-2-cases-of-refractory-t-cell-large-granular-lymphocytic-leukemia
#14
JOURNAL ARTICLE
X X Li, J P Li, K Zhou, X Zhao, F K Zhang
No abstract text is available yet for this article.
August 14, 2023: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://read.qxmd.com/read/37788085/t-cell-large-granular-lymphocytic-leukemia-in-vietnam-using-microscope-to-screen-videoconferencing-to-improve-diagnosis
#15
JOURNAL ARTICLE
Vanessa J Dayton, Dang Hoang Thien, Huynh Thien Ngon, Cade Arries, Nguyen Ngoc Sang, Nguyen Phuong Lien, Scott Kinder, Phu Chi Dung
OBJECTIVES: Leukemia diagnosis in Vietnam is limited by a lack of hematopathology training and expert consultation as well as the cost of high-magnification digitization of hematology slides. Screen-sharing software allows international collaboration with experienced hematopathologists for improved diagnostic accuracy. METHODS: A hematopathology education and consultation program was proposed for Vietnam hospitals. By appointment, pathologists in Vietnam with access to a microscope camera, imaging software, and high-speed internet were invited to review slides and data with a volunteer board-certified hematopathologist in the United States using secure videoconferencing software...
October 3, 2023: American Journal of Clinical Pathology
https://read.qxmd.com/read/37749039/-efficacy-and-safety-of-tbi-ratg-based-conditioning-regimen-for-haploidentical-allogeneic-hematopoietic-stem-cell-transplantation-in-11-cases-of-chemotherapy-resistant-advanced-peripheral-t-cell-lymphoma
#16
JOURNAL ARTICLE
R Xu, S Li, H X Liu, D L Wei, Y Jiang, J J Wang, S S Liu, C Wang, J Zhu
Objective: To evaluate the clinical outcomes and safety of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) using a conditioning regimen based on total body irradiation (TBI) and rabbit anti-human thymocyte globulin (rATG) in the management of chemotherapy-resistant advanced peripheral T-cell lymphoma (PTCL) . Methods: Clinical data of 11 patients with chemotherapy-resistant advanced PTCL who underwent haplo-HSCT with a TBI+rATG-based conditioning regimen at the Department of Hematology, Shanghai Liquan Hospital and Shanghai Zhaxin Integrated Traditional Chinese and Western Medicine Hospital, from September 2019 to December 2022 were retrospectively analyzed...
July 14, 2023: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://read.qxmd.com/read/37673632/-large-granular-lymphocytic-leukemia-and-its-association-with-immune-dysregulation
#17
JOURNAL ARTICLE
Fumihiro Ishida
Large granular lymphocytic (LGL) leukemia is a chronic lymphoproliferative disease of cytotoxic T cells or NK cells with LGL morphology and frequently complicated cytopenia and/or different autoimmune diseases, which often require medical interventions, although LGL leukemia itself is seldom lethal. Immunologic dysregulations in LGL leukemia contribute to the development of complications, for example, neutropenia with the involvement of Fas ligand system and, in pure red cell aplasia, which is a common complication among the patients of East Asian origin, impairing erythroid developments by cytotoxic T cells...
2023: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://read.qxmd.com/read/37646851/phenotype-control-techniques-for-boolean-gene-regulatory-networks
#18
REVIEW
Daniel Plaugher, David Murrugarra
Modeling cell signal transduction pathways via Boolean networks (BNs) has become an established method for analyzing intracellular communications over the last few decades. What's more, BNs provide a course-grained approach, not only to understanding molecular communications, but also for targeting pathway components that alter the long-term outcomes of the system. This has come to be known as phenotype control theory. In this review we study the interplay of various approaches for controlling gene regulatory networks such as: algebraic methods, control kernel, feedback vertex set, and stable motifs...
August 30, 2023: Bulletin of Mathematical Biology
https://read.qxmd.com/read/37646099/large-granular-lymphocyte-lymphoma-with-leukemic-phase-and-suspicion-of-leptomeningeal-lymphomatosis-in-a-cat-a-case-report
#19
N Mauri, F Graf, S Dressel-Böhm, G Scharf
In this case report we present a feline large granular lymphocyte (LGL) lymphoma, a rare morphologically distinct subtype of lymphoma, in a twelve-year-old female spayed domestic short hair cat, with high suspicion of leptomeningeal lymphomatosis due to magnetic resonance imaging findings and results of cerebral spinal fluid analyses. Diagnosis of LGL lymphoma was confirmed by means of blood cytology and polymerase chain reaction for antigen receptor rearrangements.
September 2023: Schweizer Archiv Für Tierheilkunde
https://read.qxmd.com/read/37575786/t-cell-large-granular-lymphocytic-leukemia-and-felty-syndrome-in-rheumatoid-arthritis-a-case-report
#20
Supritha Prasad, Iman Mushfiq Farooqui, Lujain AlZoubi, Shiva Arami
T-cell large granular lymphocytic (LGL) leukemia is characterized by a clonal proliferation of CD3+ T-cells and has been associated with rheumatoid arthritis (RA). Splenomegaly is a common finding and a majority of cases present with cytopenia. Felty syndrome (FS) is characterized by neutropenia and splenomegaly and is also classically described in the literature for its association with RA. Similarities in clinical features, pathogenesis, management, genetics, and immunologic basis of FS and T-cell LGL leukemia have led to the suggestion that they exist on the same spectrum of disease...
July 2023: Curēus
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