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LGL Leukemia

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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
#1
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...
October 13, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27715403/vitamin-d-decreases-stat-phosphorylation-and-inflammatory-cytokine-output-in-t-lgl-leukemia
#2
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
#3
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...
September 29, 2016: 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
#4
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
#5
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
#6
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)...
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/26252351/sphingosine-kinase-inhibitors-decrease-viability-and-induce-cell-death-in-natural-killer-large-granular-lymphocyte-leukemia
#15
Francis R LeBlanc, Xin Liu, Jeremy Hengst, Todd Fox, Valerie Calvert, Emanuel F Petricoin, Jong Yun, David J Feith, Thomas P Loughran
Sphingolipid metabolism has been identified as a potential therapeutic target in cancer. Sphingosine-1-phosphate (S1P) is a potent bioactive sphingolipid metabolite produced by sphingosine kinases-1 and -2 (SPHK1 and SPHK2). Elevated SPHK1 has been found in numerous cancer types and been shown to contribute to survival, chemotherapeutic resistance and malignancy. However, its role in large granular Natural Killer (NK) large granular lymphocyte (LGL) leukemia has not been investigated. Here, we examine SPHK1 as a therapeutic target in LGL leukemia...
2015: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/26222587/gamma-heavy-chain-disease-associated-with-large-granular-lymphocytic-leukemia-a-report-of-two-cases-and-review-of-the-literature
#16
Anais Wahbi, Antoine Neel, Francois Perrin, Julie Graveleau, Beatrice Mahe, Thomas Dejoie, Mohamed Hamidou
OBJECTIVE AND IMPORTANCE: Gamma heavy chain diseases (γHCD) and large granular lymphocyte (LGL) leukemia are two rare lymphoproliferative diseases, respectively with B and T phenotype. Both γHCD and LGL leukemia share some similar clinical features, such as cytopenias, splenomegaly, and recurrent infections. Association of these two diseases is exceptional and suggest pathogenic link. We report two cases of γHCD associated with T-LGL leukemia. CLINICAL PRESENTATION: Patient 1 was a 70-year-old woman, with lymphoplasmacytic lymphoma, refractory to chlorambucil-rituximab treatment...
March 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/26199174/recurrent-alterations-of-tnfaip3-a20-in-t-cell-large-granular-lymphocytic-leukemia
#17
Patricia Johansson, Anke Bergmann, Sven Rahmann, Inken Wohlers, René Scholtysik, Martina Przekopowitz, Marc Seifert, Gertraud Tschurtschenthaler, Gerald Webersinke, Ulrich Jäger, Reiner Siebert, Ludger Klein-Hitpass, Ulrich Dührsen, Jan Dürig, Ralf Küppers
The pathogenesis of T-cell large granular lymphocytic leukemia (T-LGL) is poorly understood, as STAT3 mutations are the only known frequent genetic lesions. Here, we identified non-synonymous alterations in the TNFAIP3 tumor suppressor gene in 3 of 39 T-LGL. In two cases these were somatic mutations, in one case the somatic origin was likely. A further case harbored a SNP that is a known risk allele for autoimmune diseases and B cell lymphomas. Thus, TNFAIP3 mutations represent recurrent genetic lesions in T-LGL that affect about 8% of cases, likely contributing to deregulated NF-κB activity in this leukemia...
January 1, 2016: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/26152552/paroxysmal-nocturnal-hemoglobinuria-pnh-and-t-cell-large-granular-lymphocyte-lgl-leukemia-an-unusual-association-another-cause-of-cytopenia-in-pnh
#18
LETTER
Thomas Boyer, Nathalie Grardel, Marie-Christine Copin, Christophe Roumier, Aurore Touzart, Anne-Laure Buchdahl, Sélim Corm, Régis Peffault de Latour, Claude Preudhomme, Louis Terriou
No abstract text is available yet for this article.
October 2015: Annals of Hematology
https://www.readbyqxmd.com/read/26071471/spectrum-of-clonal-large-granular-lymphocytes-lgls-of-%C3%AE-%C3%AE-t-cells-t-cell-clones-of-undetermined-significance-t-cell-lgl-leukemias-and-t-cell-immunoclones
#19
Timothy P Singleton, Bin Yin, Andinet Teferra, Jenny Z Mao
OBJECTIVES: Clones of T-cell large granular lymphocytes (LGLTs) were detected by flow cytometry. Disease associations are described. METHODS: Flow cytometry on blood or marrow detected clonal LGLTs by analyzing variable regions of the T-cell receptor β chain. RESULTS: LGLT clones were detected in 20% (54/264) of tested patients. The clone sizes were less than 2.0 × 10(9)/L in the blood in 73% and less than 10% of marrow space in 94%. Blood counts showed cytopenias...
July 2015: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/26064133/disseminated-gastric-malt-lymphoma-with-monoclonal-gammopathy-t-11-18-q21-q21-and-subsequent-development-of-t-large-granular-lymphocytic-leukemia-a-case-report-and-review-of-the-literature
#20
Riad Akoum, Wassim Serhal, Hussein Farhat
Background. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) is a well-characterized entity that may share clinical and morphological findings with other low-grade non-Hodgkin's lymphomas. Dissemination of MALT-type lymphoma to bone marrow and peripheral blood simultaneously with the presence of T-large granular cell leukemia (T-LGL) has rarely been reported. Case Presentation. This is the case of a 42-year-old male who presented with a gastric MALT-type lymphoma, disseminated to the bone marrow and the peripheral blood with high serum IgM levels and t(11;18)(q21;q21)...
2015: Case Reports in Medicine
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