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Large granular lymphocyte

Bryan Briney, Anne Inderbitzin, Collin Joyce, Dennis R Burton
In principle, humans can produce an antibody response to any non-self-antigen molecule in the appropriate context. This flexibility is achieved by the presence of a large repertoire of naive antibodies, the diversity of which is expanded by somatic hypermutation following antigen exposure1 . The diversity of the naive antibody repertoire in humans is estimated to be at least 1012 unique antibodies2 . Because the number of peripheral blood B cells in a healthy adult human is on the order of 5 × 109 , the circulating B cell population samples only a small fraction of this diversity...
January 21, 2019: Nature
Apostolia Papalexandri, Maria Karypidou, Evangelia Stalika, Konstantina Kotta, Tasoula Touloumenidou, Panagiota Zerva, Angeliki Paleta, Despina Mallouri, Ioannis Batsis, Ioanna Sakellari, Ioannis Kotsianidis, Achilles Anagnostopoulos, Anastasia Hadzidimitriou, Dimitris Margaritis, Kostas Stamatopoulos
Rituximab is known to affect T cell immune responses. We and others have reported expansions of T large granular lymphocytes (T-LGLs) in lymphoma patients after Rituximab. We report here the immunogenetic profiling of the T cell receptor (TR) gene repertoire in 14 patients who received Rituximab post allo-HCT and explore clinicobiological correlations. All experienced antigenic triggers, CMV, EBV re-activation and chronic GvHD and had been treated with Rituximab. Skewing of TRBV genes was observed: 3 TRBV genes accounted for half of the repertoire...
January 17, 2019: Leukemia & Lymphoma
Julie Fu, Lisa X Lee, Ping Zhou, Teresa Fogaren, Cindy Varga, Raymond L Comenzo
BACKGROUND T-cell large granular lymphocytic leukemia (T-LGL) is a rare hematological malignancy that currently has no standard therapy. Immunoglobulin heavy chain amyloidosis (AH) involving the kidney is a rare condition and the pathology, diagnosis, clinical characteristics, and prognosis are becoming understood. This report is of a rare case of T-LGL associated with renal AH and discusses the approach to management. CASE REPORT A 57-year-old woman presented with symptoms of fatigue and she had proteinuria...
January 11, 2019: American Journal of Case Reports
Raja Senthil, Arun Visakh R, Thara Pratap, Pushpa Mahadevan, V P Gangadharan
T-cell large granular lymphocytic leukemia is a rare form of leukemia, caused by clonal proliferation of cytotoxic T-cells, characterized by modest lymphocytosis and cytopenias of other lineage with hepatosplenomegaly and relatively rare lymph nodal involvement. Involvement of other organs is extremely rare. It is predominantly an indolent disease and most of patients remain asymptomatic for a long period. We present a rare case of aggressive form (CD56 positive) of large granular lymphocytic leukemia with atypical presentations mimicking pleural malignancy on F-FDG PET/CT...
January 4, 2019: Clinical Nuclear Medicine
Shosaku Nomura, Tomoki Ito, Atsushi Satake, Kazuyoshi Ishii
Background: The outcome for chronic myelogenous leukemia (CML) patients presented in the chronic phase has changed dramatically since the introduction of tyrosine kinase inhibitor (TKI) therapy. Notably, an increased incidence of large granular lymphocytes (LGLs), which is related to immunological conditions, appears to be predictive of a favorable outcome for dasatinib therapy. We therefore examined the immunological characteristics of CML patients during dasatinib therapy by determining the plasma concentrations of five different biomarkers...
2019: Journal of Blood Medicine
Maya Belhadj, Dalila Mansour, Sophie Kaltenbach, Benedicte Deau-Fischer, Patricia Franchi, Jérôme Tamburini, Nicolas Chapuis, Diane Damotte, Olivier Kosmider, Barbara Burroni, Didier Bouscary
No abstract text is available yet for this article.
December 20, 2018: Haematologica
Michael E Kallen, Alina Dulau-Florea, Weixin Wang, Katherine R Calvo
Bone marrow failure and related syndromes are rare disorders characterized by ineffective bone marrow hematopoiesis and peripheral cytopenias. Although many are associated with characteristic clinical features, recent advances have shown a more complicated picture with a spectrum of broad and overlapping phenotypes and imperfect genotype-phenotype correlations. Distinguishing acquired from inherited forms of marrow failure can be challenging, but is of crucial importance given differences in the risk of disease progression to myelodysplastic syndrome, acute myeloid leukemia, and other malignancies, as well as the potential to genetically screen relatives and select the appropriate donor if hematopoietic stem cell transplantation becomes necessary...
January 2019: Seminars in Hematology
Mayumi Hatsuse, Yoko Taminishi, Saori Maegawa-Matsui, Shin-Ichi Fuchida, Akira Okano, Satoshi Murakami, Toru Inaba, Chihiro Shimazaki
A 61-year-old female was diagnosed with a lymphoid crisis of chronic myeloid leukemia (CML) in February 201X and started chemotherapy combined with dasatinib (DAS). After 1 month of initiating second consolidation therapy, the neutrophils decreased to 1%, bone marrow examination revealed large granular lymphocytes (LGL) at 13%, and complete cytogenetic remission was attained (CCyR). Suspecting DAS-induced agranulocytosis, DAS was discontinued. After 2 weeks, LGL disappeared and neutrophils recovered. In this case, CCyR was attained for the first time when LGL increased...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Kristine C Olson, Paige M Kulling Larkin, Rossana Signorelli, Cait E Hamele, Thomas L Olson, Mark R Conaway, David J Feith, Thomas P Loughran
Calcitriol, the active form of vitamin D, has been well documented to act directly on immune cells and malignant cells. Activated T cells are one of the best characterized targets of calcitriol, with effects including decreasing inflammatory cytokine output and promoting anti-inflammatory cytokine production. However, the effects of calcitriol on natural killer (NK) cells are less clear. Reports suggest that only immature NK cell populations are affected by calcitriol treatment resulting in impaired cytotoxic function and cytokine production, while mature NK cells may have little or no response...
November 16, 2018: Cytokine
C Suzuki, I Hirai, H Nomura, T Ouchi, M Okayama, S Okamoto, M Amagai, K Tanese, H Takahashi
T cell large granular lymphocyte leukemia (T-LGLL) is a rare chronic lymphoproliferative disorder of mature post-thymic large granular lymphocytes (LGL); either cytotoxic T-lymphocytes or natural killer cells.1 Major phenotype of LGL cells is TCRαβ+CD3+CD4-CD5+CD8+CD27-CD28-CD45-CD57+.2 Less than 10% of cases express TCRγδ instead of TCRαβ.2 Most cases of T-LGLL have an indolent course1 and its cutaneous manifestations have been poorly documented. This article is protected by copyright. All rights reserved...
November 16, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Sandra Kojić Katović, Ankica Vasilj, Goran Rinčić
T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Here we present a case of a 53-year-old male admitted to the hospital because of abdominal pain...
June 2018: Acta Clinica Croatica
Zhi-Yuan Qiu, Yan Wang, Jun Zhang, Zhao Zhang, Lei Fan, Rong Wang, Yu-Jie Wu, Ye-Qing Zhang, Wei Xu, Jian-Yong Li
RATIONALE: T-cell large granular lymphocytic leukemia (T-LGLL) is a rare lymphoproliferative neoplasm of cytotoxic T cells and renal cell carcinoma (RCC) is the most common form of kidney cancer, but T-LGLL associated with RCC has never been reported. PATIENT CONCERNS: A 58-year-old Chinese male presented with general fatigue and intermittent-remittent fever, accompanied by palpitations and dizziness. DIAGNOSIS: Radical nephrectomy was performed, and a diagnosis of clear cell carcinoma (T1N0M0, I phase) was made based on the postoperative pathology findings...
November 2018: Medicine (Baltimore)
A G Vakrakou, D Tzanetakos, S Valsami, E Grigoriou, K Psarra, J Tzartos, M Anagnostouli, E Andreadou, M E Evangelopoulos, G Koutsis, C Chrysovitsanou, E Gialafos, A Dimitrakopoulos, L Stefanis, C Kilidireas
BACKGROUND: Alemtuzumab has been demonstrated to reduce the risks of relapse and accumulation of sustained disability in Multiple Sclerosis (MS) patients compared to β-interferon. It acts against CD52, leading primarily to lymphopenia. Recent data have shown that mild neutropenia is observed in 16% of treated MS-patients whereas severe neutropenia occurred in 0.6%. CASE PRESENTATION: Herein, we present the case of a 34-year-old woman with relapsing-remitting MS, with a history of treatment with glatiramer acetate and natalizumab, who subsequently received Alemtuzumab (12 mg / 24 h × 5 days)...
October 29, 2018: BMC Neurology
Fumihiro Ishida
Aggressive NK cell leukemia (ANKL) is a rare malignant lymphoproliferative disorder of mature NK cells closely associated with Epstein-Barr virus (EBV) and more common in East Asia than in other areas. Significant variations exist in the morphology of ANKL tumor cells, from typical large granular lymphocyte morphology to highly atypical features with basophilic cytoplasm containing azurophilc granules. The main involved sites are hepatosplenic lesions, bone marrow and peripheral blood, and nasal or skin lesions are infrequent...
2018: Frontiers in Pediatrics
T Tiffany Wang, Jun Yang, Yong Zhang, Meili Zhang, Sigrid Dubois, Kevin C Conlon, Yutaka Tagaya, Cait E Hamele, Shubha Dighe, Thomas L Olson, David J Feith, Nazli Azimi, Thomas A Waldmann, Thomas P Loughran
Interleukin-15 (IL-15) and IL-2 drive T-cell malignancies including T-cell large granular lymphocyte leukemia (T-LGLL) and HTLV-1 driven adult T-cell leukemia (ATL). Both cytokines share common γ-chain receptors and downstream signaling pathways. T-LGLL is characterized by clonal expansion of cytotoxic T cells and is associated with abnormal JAK/STAT signaling. ATL is an aggressive CD4+ T-cell neoplasm associated with HTLV-1. T-LGLL and ATL share dependence on IL-2 and IL-15 for survival and both diseases lack effective therapies...
October 23, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Toru Kawakami, Nodoka Sekiguchi, Jun Kobayashi, Tatsuya Imi, Kazuyuki Matsuda, Taku Yamane, Sayaka Nishina, Yasushi Senoo, Hitoshi Sakai, Toshiro Ito, Tomonobu Koizumi, Makoto Hirokawa, Shinji Nakao, Hideyuki Nakazawa, Fumihiro Ishida
Dysregulation of T-cell-mediated immunity is responsible for acquired pure red cell aplasia (PRCA). Although STAT3 mutations are frequently detected in patients with T-cell large granular lymphocytic leukemia (T-LGLL), which is often complicated by PRCA and which is also reported to be associated with acquired aplastic anemia (AA) and myelodysplastic syndrome (MDS), whether STAT3 -mutated T cells are involved in the pathophysiology of PRCA and other types of bone marrow failure remains unknown. We performed STAT3 mutation analyses of the peripheral blood mononuclear cells from PRCA patients (n = 42), AA (n = 54), AA-paroxysmal nocturnal hemoglobinuria (AA-PNH; n = 7), and MDS (n = 21) using an allele-specific polymerase chain reaction and amplicon sequencing...
October 23, 2018: Blood Advances
Houfang Sun, Sheng Wei, Lili Yang
OBJECTIVES: Large granular lymphocyte (LGL) leukemia is a rare type of lymphoproliferative disease caused by clonal antigenic stimulation of T cells and natural killer (NK) cells. METHODS: In this review, we focus on the current knowledge of the immunological dysfunctions associated with LGL leukemia and the associated disorders coexistent with this disease. Novel therapeutic options targeting known molecular mechanisms are also discussed. RESULTS AND DISCUSSION: The pathogenesis of LGL leukemia involves the accumulation of gene mutations, dysregulated signaling pathways and immunological dysfunction...
December 2019: Hematology (Amsterdam, Netherlands)
Suheyla Ekemen, Ant Uzay, Nuray Bassullu, Emel Dikicioglu-Cetin, Kyoko Matsuda, Umit Ince, Cevayir Coban
BACKGROUND: Malaria is known to cause acute and deadly complications. However, malaria can cause unforeseen pathologies due to its chronicity. It increases the risk of endemic Burkitt Lymphoma development by inducing DNA damage in germinal centre (GC) B cells, and leading higher frequency of Epstein-Barr virus (EBV)-infected cells in GCs. EBV is well known for its tropism for B cells. However, less is known about EBV's interaction with T cells and its association with T cell lymphoma...
October 5, 2018: Malaria Journal
Aya Nakaya, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yoshiko Azuma, Yukie Tsubokura, Masaaki Hotta, Hideaki Yoshimura, Kazuyoshi Ishii, Tomoki Ito, Shosaku Nomura
Dasatinib is currently approved for clinical use as a first-line treatment agent for newly diagnosed chronic myeloid leukemia (CML). However, only a few clinical trials have been performed to evaluate dasatinibinduced PE following first-line therapy. We investigated the incidence and clinical features of dasatinib-induced PE following first-line therapy in Japanese CML patients of real world clinical practice settings. Among 22 patients, the median age of PE-positive patients was higher than that of PE-negative patients...
September 5, 2018: Hematology Reports
Isao Matsumoto, Ko Nakashima, Yuko Goto-Koshino, James Kenn Chambers, Hajime Tsujimoto, Hiroyuki Nakayama, Kazuyuki Uchida
Human enteropathy-associated T-cell lymphoma (EATL) is classified into 2 distinct subgroups based on their phenotypes (type I and type II). Canine intestinal T-cell lymphoma can be morphologically classified into large and small cell lymphomas (LCL and SCL, respectively). Their association with human EATL or immunohistochemical and biological features has not been well characterized. In this study, the immunohistochemical profiles of 17 cases of LCL and 33 cases of SCL were evaluated with markers used for human EATL classification...
October 3, 2018: Veterinary Pathology
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