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Large granular lymphocyte leukemia

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https://www.readbyqxmd.com/read/27896231/prolonged-complete-hematologic-response-in-relapsed-refractory-t-large-granular-lymphocyte-leukemia-after-bendamustine-treatment
#1
R Rosamilio, V Giudice, I Ferrara, S Annunziata, L Pezzullo, G Villani, C Baldi, R Guariglia, M Rocco, C Selleri
T-large granular lymphocyte leukemia (T-LGLL) is a chronic clonal proliferation of effector memory cytotoxic CD3(+)CD57(+)CD56(-) T cells and the current guidelines suggest immunosuppressive therapy as first-line therapy, but the treatment of refractory/relapsed patients is still challenging due to the lack of prospective studies. We describe a series of two refractory/relapsed T-LGLL patients successfully treated with bendamustine, a chemotherapeutic agent largely used for B-cell neoplasms, but poorly investigated for the treatment of T-cell diseases...
November 2016: Translational Medicine @ UniSa
https://www.readbyqxmd.com/read/27881371/pure-red-cell-aplasia
#2
REVIEW
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27867335/pyoderma-gangrenosum-in-the-urologist-clinic
#3
Dina J Ludwig, Hossain Roshani, Martijn G Steffens, Frederik C Moll, Robertus G Teepe
Pyoderma gangrenosum is a rare non-infectious skin disorder. It is often associated with systemic diseases, like the inflammatory bowel disease, rheumatological disease and (hematological) malignancy. The diagnosis is affirmed through a process of elimination and is principally based on clinical presentation and course. We present a 59-year-old male with T-cell large granular lymphocyte leukemia and pyoderma gangrenosum of penis and scrotum. Finally the patient was successfully treated with systemic prednisolone...
October 2016: Current Urology
https://www.readbyqxmd.com/read/27830541/achievement-of-disease-control-with-donor-derived-eb-virus-specific-cytotoxic-t-cells-after-allogeneic-peripheral-blood-stem-cell-transplantation-for-aggressive-nk-cell-leukemia
#4
Shojiro Haji, Motoaki Shiratsuchi, Takamitsu Matsushima, Akiko Takamatsu, Mariko Tsuda, Yasuhiro Tsukamoto, Emi Tanaka, Hirofumi Ohno, Eriko Fujioka, Yuriko Ishikawa, Ken-Ichi Imadome, Yoshihiro Ogawa
Aggressive NK-cell leukemia (ANKL) is characterized by systemic infiltration of Epstein-Barr virus (EBV)-associated natural killer cells and poor prognosis. We report a case of ANKL in which EBV-specific cytotoxic T lymphocytes (CTLs) were induced. A 41-year-old male suffered from fever, pancytopenia, and hepatosplenomegaly. The number of abnormal large granular lymphocytes in the bone marrow was increased and the cells were positive for CD56 and EBV-encoded small nuclear RNAs. The patient was diagnosed with ANKL and achieved a complete response following intensive chemotherapy...
November 9, 2016: International Journal of Hematology
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
#5
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
#6
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/27697773/high-incidence-of-activating-stat5b-mutations-in-cd4-positive-t-cell-large-granular-lymphocyte-leukemia
#7
Emma Andersson, Takahiro Tanahashi, Nodoka Sekiguchi, Vanessa Rebecca Gasparini, Sabrina Bortoluzzi, Toru Kawakami, Kazuyuki Matsuda, Takeki Mitsui, Samuli Eldfors, Stefania Bortoluzzi, Alessandro Coppe, Andrea Binatti, Sonja Lagström, Pekka Ellonen, Noriyasu Fukushima, Sayaka Nishina, Noriko Senoo, Hitoshi Sakai, Hideyuki Nakazawa, Yok-Lam Kwong, Thomas P Loughran, Jaroslaw P Maciejewski, Satu Mustjoki, Fumihiro Ishida
No abstract text is available yet for this article.
October 3, 2016: Blood
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
#8
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/27662842/identification-of-control-targets-in-boolean-molecular-network-models-via-computational-algebra
#9
David Murrugarra, Alan Veliz-Cuba, Boris Aguilar, Reinhard Laubenbacher
BACKGROUND: Many problems in biomedicine and other areas of the life sciences can be characterized as control problems, with the goal of finding strategies to change a disease or otherwise undesirable state of a biological system into another, more desirable, state through an intervention, such as a drug or other therapeutic treatment. The identification of such strategies is typically based on a mathematical model of the process to be altered through targeted control inputs. This paper focuses on processes at the molecular level that determine the state of an individual cell, involving signaling or gene regulation...
September 23, 2016: BMC Systems Biology
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/27542218/methotrexate-therapy-of-t-cell-large-granular-lymphocytic-leukemia-impact-of-stat3-mutation
#11
Zhi-Yuan Qiu, Lei Fan, Rong Wang, Robert Peter Gale, Hua-Jin Liang, Man Wang, Li Wang, Yu-Jie Wu, Chun Qiao, Yao-Yu Chen, Wei Xu, Jun Qian, Jian-Yong Li
T-cell large granular lymphocytic leukemia (T-LGLL) is a rare haematologic neoplasm. Consequntly, there are no large prospective studies of therapy and no uniform therapy recommendations. We analyzed data from 36 subjects receiving methotrexate alone (N = 27) or with prednisone (N = 9) as initial therapy. 31 subjects responded (86%, 95% confidence interval [CI], 73, 95%) with 8 complete responses and 23 partial responses. Median time-to-response was 3 months (range, 1-5 months). Median response duration was 20 months (range, 2-55 months)...
August 17, 2016: Oncotarget
https://www.readbyqxmd.com/read/27496109/donor-derived-t-cell-large-granular-lymphocytic-leukemia-in-a-patient-with-peripheral-t-cell-lymphoma
#12
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
#13
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/27469326/cyclin-d1-expression-in-peripheral-t-cell-lymphomas
#14
Joo Y Song, Liping Song, Alex F Herrera, Girish Venkataraman, Joyce L Murata-Collins, Victoria H Bedell, Yuan Yuan Chen, Young S Kim, Reda Tadros, Bharat N Nathwani, Dennis D Weisenburger, Andrew L Feldman
Cyclin D1 is an important regulator of the cell cycle and overexpression of this protein by immunohistochemistry is characteristically seen in mantle cell lymphoma and other B-cell neoplasms. However, little is known about the expression of this protein in T-cell lymphomas. Cyclin-dependent kinase pathway inhibitors are in development, therefore identifying cyclin D1-positive T-cell lymphomas may provide a therapeutic target in a disease where novel treatments are urgently needed. We collected 200 peripheral T-cell lymphomas from three institutions including the following types of cases: 34 anaplastic large cell lymphoma, ALK+, 44 anaplastic large cell lymphoma, ALK negative, 68 peripheral T-cell lymphomas, not otherwise specified, 24 angioimmunoblastic T-cell lymphomas, 7 extranodal NK/T-cell lymphomas, 4 enteropathy associated T-cell lymphomas, 3 hepatosplenic T-cell lymphomas, 12 cutaneous T-cell lymphomas, and 4 large granular lymphocytic leukemias...
July 29, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27438095/characteristics-of-lymphoproliferative-disorders-with-more-than-one-aberrant-cell-population-as-detected-by-10-color-flow-cytometry
#15
Talal Mahdi, Amr Rajab, Ruth Padmore, Anna Porwit
BACKGROUND: We have evaluated the frequency of lymphoproliferative disorders with more than one aberrant population of monotypic B-cells detected during routine hematopathological diagnostics. MATERIALS AND METHODS: 2600 samples peripheral (blood, bone marrow, fine needle aspirate, lymph node and pleural fluid cell suspensions) were analyzed using a ten-color B-cell panel and a ten-color T-cell panel. A ten-color plasma cell/lymphoplasmacytic panel was performed when appropriate...
July 20, 2016: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/27408372/cd56-negative-aggressive-t-cell-large-granular-lymphocytic-leukemia
#16
Mary Theresa Sylvia, Sajini Elizabeth Jacob, Debdatta Basu, Deepak Amalnath, Tarun Kumar Dutta
T cell large granular lymphocytic leukemia is a clonal proliferation of cytotoxic large granular T cells positive for CD3 and CD8. It is a chronic lymphoproliferative disorder with an indolent course. Therapeutic options include observation and low dose chemotherapy. Rarely, they have an aggressive course. Such cases have expression of NK cell associated antigens like CD56 in the T cells. These cases require more aggressive therapy with acute lymphoblastic leukemia regimens. We report a case of fatal CD56 negative T cell large granular lymphocytic leukemia in a 38 year old lady...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27349810/principal-component-analysis-uncovers-cytomegalovirus-associated-nk-cell-activation-in-ph-leukemia-patients-treated-with-dasatinib
#17
K-I Ishiyama, T Kitawaki, N Sugimoto, T Sozu, N Anzai, M Okada, M Nohgawa, K Hatanaka, N Arima, T Ishikawa, S Tabata, T Onaka, S Oka, Y Nakabo, R Amakawa, M Matsui, T Moriguchi, A Takaori-Kondo, N Kadowaki
Dasatinib treatment markedly increases the number of large granular lymphocytes (LGLs) in a proportion of Ph(+) leukemia patients, which associates with a better prognosis. The lymphocytosis is predominantly observed in cytomegalovirus (CMV)-seropositive patients, yet detectable CMV reactivation exists only in a small fraction of patients. Thus, etiology of the lymphocytosis still remains unclear. Here, we identified NK cells as the dominant LGLs expanding in dasatinib-treated patients, and applied principal component analysis (PCA) to an extensive panel of NK cell markers to explore underlying factors in NK cell activation...
June 28, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27342492/-analysis-of-clinical-characteristics-in-10-patietns-with-t-large-granular-lymphocytic-leukemia
#18
An-Qi Liu, Lei Zhou, Yong-Hui Li, Yu Jing, Shu-Hong Wang, Jun-Hui Mei, Li-Ping Dou, Li-Li Wang, Li Yu
OBJECTIVE: To analyze the clinical manifestations and laboratory features of patients with T large granular lymphocytic leukemia (T-LGLL), so as to improve the understanding of this disease. METHODS: The clinical data of 10 patients with T-LGLL in General Hospital of Chinese PLA from October 2015 to March 2010 were analyzed retrospectively. RESULTS: Their median age at diagnosis was 51 years old. 9/10 (90%) patients showed symptoms of anemia, with a median Hb level of 82...
June 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
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
#19
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/27077768/moderate-dose-cyclophosphamide-in-the-treatment-of-relapsed-refractory-t-cell-large-granular-lymphocytic-leukemia-associated-pure-red-cell-aplasia
#20
Guangxin Peng, Wenrui Yang, Li Zhang, Kang Zhou, Yang Li, Yuan Li, Lei Ye, Jianping Li, Huihui Fan, Lin Song, Xin Zhao, Zhijie Wu, Fengkui Zhang, Liping Jing
BACKGROUND: T-cell large granular lymphocyte leukemia (T-LGLL) is a rare disorder characterized by clonal proliferation of large granular lymphocytes (commonly CD3+/CD8+/CD57+). However, the available data regarding the optimal treatment for relapsed/refractory T-LGLL patients are limited. METHODS: We retrospectively reviewed 10 patients treated with immunosuppressive therapy consisting of intravenous moderate-dose cyclophosphamide (MD-CTX) together with oral cyclosporine A for relapsed/refractory T-LGLL in our hospital between July 2006 and March 2013...
April 2016: Hematology (Amsterdam, Netherlands)
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