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LGL Leukemia

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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
#1
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...
February 27, 2017: Oncotarget
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
#2
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
#3
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/28128670/large-granular-lymphocytic-leukemia-current-diagnostic-and-therapeutic-approaches-and-novel-treatment-options
#4
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
#5
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/27932186/late-graft-rejection-in-association-with-t-large-granular-lymphocyte-expansion-of-recipient-origin-after-human-leukocyte-antigen-haploidentical-stem-cell-transplantation-a-case-report
#6
N Nakagawa, H Yamazaki, G Aoki, Y Kondo, S Nakao
BACKGROUND: Large granular lymphocyte (LGL) expansion occasionally occurs after allogeneic stem cell transplantation (allo-SCT), and is thought to be a good prognostic sign that is associated with a lower relapse rate. However, there have been no reports of late graft failure (LGF) due to graft rejection in association with oligoclonal LGL expansion. We herein report a case of LGF associated with the transient expansion of recipient-derived T-LGL after allo-SCT. CASE REPORT: A 65-year-old man underwent peripheral blood stem cell transplantation (PBSCT) from his human leukocyte antigen (HLA)-haploidentical son for the treatment of acute myeloid leukemia, which had evolved from a myelodysplastic syndrome (MDS)...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27755009/distinguishing-between-hepatosplenic-t-cell-lymphoma-and-%C3%AE-%C3%AE-t-cell-large-granular-lymphocytic-leukemia-a-clinicopathologic-immunophenotypic-and-molecular-analysis
#7
Mariko Yabe, L Jeffrey Medeiros, Sa A Wang, Guilin Tang, Carlos E Bueso-Ramos, Jeffrey L Jorgensen, Govind Bhagat, Weina Chen, Shaoying Li, Ken H Young, Roberto N Miranda
Hepatosplenic T-cell lymphoma (HSTCL) is a rare, aggressive T-cell lymphoma that can be challenging to diagnose. In particular, distinguishing HSTCL from T-cell large granular lymphocytic (T-LGL) leukemia of γδ T-cell receptor (TCR) type is difficult without examination of a splenectomy specimen. In this study, we systematically assessed a series of HSTCL cases for findings reported in the literature as supporting or not supporting the diagnosis of HSTCL. We also compared HSTCL with a group of cases of T-LGL of γδ TCR type...
January 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27715403/vitamin-d-decreases-stat-phosphorylation-and-inflammatory-cytokine-output-in-t-lgl-leukemia
#8
Kristine C Olson, Paige M Kulling, Thomas L Olson, Su-Fern Tan, Rebecca J Rainbow, David J Feith, Thomas P Loughran
Large granular lymphocyte leukemia (LGLL) is a rare incurable chronic disease typically characterized by clonal expansion of CD3+ cytotoxic T-cells. Two signal transducer and activator of transcription factors, STAT1 and STAT3, are constitutively active in T-LGLL. Disruption of this activation induces apoptosis in T-LGLL cells. Therefore, considerable efforts are focused on developing treatments that inhibit STAT activation. Calcitriol, the active form of vitamin D, has been shown to decrease STAT1 and STAT3 phosphorylation in cancer cell lines and autoimmune disease mouse models...
October 7, 2016: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/27686672/a-screening-method-with-lymphocyte-percentage-and-proportion-of-granular-lymphocytes-in-the-peripheral-blood-for-large-granular-lymphocyte-lgl-leukemia
#9
Takahiro Tanahashi, Nodoka Sekiguchi, Kazuyuki Matsuda, Akihiro Matsumoto, Toshiro Ito, Hideyuki Nakazawa, Fumihiro Ishida
Large granular lymphocyte leukemia (LGL-L) is defined morphologically as a group of lymphoproliferative disorders, including T-cell large granular lymphocytic leukemia (T-LGL-L), chronic lymphoproliferative disorders of NK cells (CLPD-NK), and aggressive NK cell leukemia. We investigated the morphological features of LGL leukemic cells in LGL-L patients to identify screening methods best suited to application in daily clinical practice. LGL leukemic cells were mostly indistinguishable from normal LGL; however, we developed a simplified approach to distinguishing among these cells, in which lymphocyte  % and the proportion of granular lymphocytes among lymphocytes (GL %) can serve as parameters at the cut-off values of 52 and 50 %, respectively...
January 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/27640075/single-center-series-of-bone-marrow-biopsy-defined-large-granular-lymphocyte-leukemia-high-rates-of-sustained-response-to-oral-methotrexate
#10
Talha Munir, Mark J Bishton, Ian Carter, Andrew McMillan, Simon O'Connor, Vishakha Sovani, Andrew P Haynes, Christopher P Fox
INTRODUCTION: Large granular lymphocyte (LGL) leukemia is a rare chronic lymphoproliferative disorder, with few large series reported to date. Series using stringent diagnostic criteria incorporating bone marrow biopsy (BMB), immunophenotyping, and T-cell receptor rearrangements are even scarcer. PATIENTS AND METHODS: The present study was a single-center series of 39 patients with LGL leukemia diagnosed using immunohistochemical analysis of BMB samples and flow cytometric and molecular data...
December 2016: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/27496109/donor-derived-t-cell-large-granular-lymphocytic-leukemia-in-a-patient-with-peripheral-t-cell-lymphoma
#11
Juliana E Hidalgo Lopez, Mariko Yabe, Adrian A Carballo-Zarate, Sa A Wang, Jeffrey L Jorgensen, Sairah Ahmed, John Lee, Shaoying Li, Ellen Schlette, Timothy McDonnell, Roberto N Miranda, L Jeffrey Medeiros, Carlos E Bueso-Ramos, C Cameron Yin
T-cell large granular lymphocytic (T-LGL) leukemia after hematopoietic stem cell transplantation (SCT) is rare and its natural history and clinical outcome have not been well described. We report the clinical, morphologic, immunophenotypic, and molecular features of a case of donor-derived T-LGL leukemia in a 16-year-old man who received allogeneic SCT for peripheral T-cell lymphoma not otherwise specified (PTCL-NOS). The patient presented with persistent neutropenia and splenomegaly 9 months after SCT when the chimerism study showed a 100% donor pattern...
August 2016: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/27494824/a-population-based-study-of-large-granular-lymphocyte-leukemia
#12
M V Shah, C C Hook, T G Call, R S Go
Large granular lymphocyte (LGL) leukemia is a lymphoproliferative disorder of cytotoxic cells. T-cell LGL (T-LGL) leukemia is characterized by accumulation of cytotoxic T cells in blood and infiltration of the bone marrow, liver or spleen. Population-based studies have not been reported in LGL leukemia. We present clinical characteristics, natural history and risk factors for poor survival in patients with LGL leukemia using the Surveillance, Epidemiology, and End Results Program (SEER) and the United States National Cancer Data Base (NCDB)...
August 5, 2016: Blood Cancer Journal
https://www.readbyqxmd.com/read/27278595/the-legacy-of-the-f344-rat-as-a-cancer-bioassay-model-a-retrospective-summary-of-three-common-f344-rat-neoplasms
#13
Robert R Maronpot, Abraham Nyska, Jennifer E Foreman, Yuval Ramot
The Fischer 344 (F344) rat was used by the National Toxicology Program (NTP) for over 5 decades for toxicity and carcinogenicity studies. However, in 2006, the NTP decided to switch to a different rat stock due largely to high background control incidences of Leydig cell tumors (LCTs) and mononuclear cell leukemia (MNCL), also known as large granular lymphocytic (LGL) leukemia. In the current review, we aim (1) to provide a summary of NTP bioassays with treatment-associated effects involving MNCL and LCTs in addition to male F344-specific tunica vaginalis mesothelioma (TVM); (2) to describe important pathobiological differences between these F344 rat tumor responses and similar target tissue-tumor response in humans; and (3) to present the NTP reasons for switching away from the F344 rat...
September 2016: Critical Reviews in Toxicology
https://www.readbyqxmd.com/read/27064362/cell-size-variations-of-large-granular-lymphocyte-leukemia-implication-of-a-small-cell-subtype-of-granular-lymphocyte-leukemia-with-stat3-mutations
#14
Takahiro Tanahashi, Nodoka Sekiguchi, Kazuyuki Matsuda, Yuka Takezawa, Toshiro Ito, Hikaru Kobayashi, Naoaki Ichikawa, Sayaka Nishina, Noriko Senoo, Hitoshi Sakai, Hideyuki Nakazawa, Fumihiro Ishida
Large granular lymphocyte leukemia (LGL-L) has been morphologically defined as a group of lymphoproliferative disorders, including T-cell large granular lymphocytic leukemia (T-LGL-L), chronic lymphoproliferative disorders of NK cells (CLPD-NK) and aggressive NK cell leukemia. We investigated the morphological features of LGL leukemic cells in 26 LGL-L patients in order to elucidate relationships with current classifications and molecular backgrounds. LGL-L cells were mostly indistinguishable from normal LGL...
June 2016: Leukemia Research
https://www.readbyqxmd.com/read/26922203/rheumatoid-arthritis-and-associated-large-granular-lymphocytic-leukemia-successful-treatment-with-rituximab
#15
A Raposo, M Cerqueira, J Costa, J Sousa Neves, F Teixeira, C Afonso
Large granular lymphocytic (LGL) leukemia is an uncommon, usually indolent, lymphoproliferative disorder strongly associated with various autoimmune diseases. The authors report a case of a 67-year-old woman with a long-standing rheumatoid arthritis, who developed neutropenia and associated recurrent infections, diagnosed with LGL leukemia. We describe the treatment approach and the response to an anti-TNF and the rituximab.
October 2015: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/26913432/-large-granular-lymphocytic-leukemia-and-jak-stat-signaling-pathway-review
#16
REVIEW
Zhi-Yuan Qiu, Yu Fan
Large granular lymphocytic leukemia (LGLL) is a rare lymphoproliferative disorder of clonal expansion of cytotoxic T- or NK-cells in blood and bone marrow, and often associated with autoimmune disorders. According to the current WHO classification of the hematopoietic and lymphoid tissue tumors, the clonal LGL expansions are further classified as T-cell large granular lymphocytic leukemia (T-LGLL), chronic lymphoproliferative disorders of NK cells (CLPD-NK) and aggressive NK cell leukemia. Since there is a general lack of awareness of this disease, some patients may be misdiagnosed or some cases may be missed when diagnosis was done...
February 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/26731886/large-granular-lymphocytic-leukemia-a-tale-of-two-lgls
#17
Shaun Hanson, Michael Guarino, Pamela Simpson
Large granular lymphocytic (LGL) leukemia is a fairly uncommon hematological malignancy, generally indolent in nature. Two patients with this entity receiving care at the Helen F. Graham Cancer Center and Research Institute (HFGCCRI) are described, who together illustrate the great range of severity that can exist in this disease. One patient, SM, presented with life-threatening anemia, yet another patient, TD, presented merely with asymptomatic lymphocytosis. Management differed accordingly, comprising methotrexate therapy for SM and active surveillance for TD...
November 2015: Delaware Medical Journal
https://www.readbyqxmd.com/read/26386082/clinicopathologic-immunophenotypic-cytogenetic-and-molecular-features-of-%C3%AE-%C3%AE-t-cell-large-granular-lymphocytic-leukemia-an-analysis-of-14-patients-suggests-biologic-differences-with-%C3%AE-%C3%AE-t-cell-large-granular-lymphocytic-leukemia-corrected
#18
Mariko Yabe, L Jeffrey Medeiros, Sa A Wang, Sergej Konoplev, Chi Young Ok, Sanam Loghavi, Gary Lu, Lauren Flores, Joseph D Khoury, R Craig Cason, Ken H Young, Roberto N Miranda
OBJECTIVES: T-cell large granular lymphocytic (T-LGL) leukemia is a rare disorder in which the neoplastic cells usually express the αβ T-cell receptor (TCR). To determine the significance of γδ TCR expression in this leukemia, we compared the clinicopathologic, immunophenotypic, and genetic features of patients with T-LGL leukemia expressing γδ TCR or αβ TCR. METHODS: We used the World Health Organization classification criteria to confirm the diagnosis...
October 2015: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/26337633/rheumatoid-arthritis-and-associated-large-granular-lymphocytic-leukemia-successful-treatment-with-rituximab
#19
Ana Raposo, Marcos Cerqueira, José Costa, Joana Sousa Neves, Filipa Teixeira, Carmo Afonso
Large granular lymphocytic (LGL) leukemia is an uncommon, usually indolent, lymphoproliferative disorder strongly associated with various autoimmune diseases. The authors report a case of a 67-year-old woman with a long-standing rheumatoid arthritis, who developed neutropenia and associated recurrent infections, diagnosed with LGL leukemia. We describe the treatment approach and the response to an anti-TNF and the rituximab.
June 18, 2015: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/26267232/the-longitudinal-analysis-of-large-granular-lymphocytosis-in-patients-with-philadelphia-chromosome-positive-leukemia-treated-with-dasatinib
#20
Yuji Shimura, Shigeo Horiike, Yasuhiko Tsutsumi, Mayumi Hatsuse, Akira Okano, Shin-Ichi Fuchida, Tsutomu Kobayashi, Yosuke Matsumoto, Junya Kuroda, Eri Kawata-Iida, Hitoji Uchiyama, Nobuhiko Uoshima, Chihiro Shimazaki, Hiroto Kaneko, Yutaka Kobayashi, Masafumi Taniwaki
Dasatinib, a 2nd-generation tyrosine kinase inhibitor (TKI), can specifically induce large granular lymphocytes (LGL) in some patients with Philadelphia chromosome (Ph)-positive leukemia. To investigate the properties of the induced LGLs, we performed prospective and longitudinal analyses. From Feb 2011 to Jan 2013, a total of 17 patients with Ph-positive leukemia who were previously untreated or refractory to imatinib were enrolled. T cell receptor (TCR)-γ/δ gene rearrangements and phenotypic profiles of lymphocytes were examined before and during administration of dasatinib...
October 2015: International Journal of Hematology
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