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NK cell chronic lymphoproliferative disorder

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https://www.readbyqxmd.com/read/29417693/mutations-in-the-stat-family-of-genes-in-cancer
#1
REVIEW
Nahid Shahmarvand, Alexandra Nagy, Jahanbanoo Shahryari, Robert S Ohgami
In recent years, it has become clear that members of the signal transducer and activator of transcription (STAT) family of genes play an important role in cancer. The STAT family consists of six genes, STAT1-4, STAT5A/ STAT5B and STAT6, that are involved in regulating cellular proliferation, apoptosis, angiogenesis and the immune system response. Constitutive activation of STAT3, via mutational changes, has been shown to be important in oncogenesis in both solid and hematopoietic cancers. In the case of hematopoietic neoplasms, STAT3 driver mutations have been described in T-cell large granular lymphocytic leukemia (T-LGL) and chronic natural killer lymphoproliferative disorders (CLPD-NK) and are seen in 30-40% of T-LGL patients...
February 8, 2018: Cancer Science
https://www.readbyqxmd.com/read/29385279/chronic-lymphoproliferative-disorder-of-nk-cells-a-single-institution-review-with-emphasis-on-relative-utility-of-multimodality-diagnostic-tools
#2
Habibe Kurt, Jeffrey L Jorgensen, Hesham M Amin, Keyur P Patel, Sa A Wang, Pei Lin, Rashmi Kanagal-Shamanna, Sanam Loghavi, Beenu Thakral, Haitham A Khogeer, Elias J Jabbour, Shaoying Li, C Cameron Yin, L Jeffrey Medeiros, Joseph D Khoury
BACKGROUND: Chronic lymphoproliferative disorder of NK-cells (CLPD-NK) manifests as a persistent increase (≥2 x 109 /L, for > 6 months) of mature NK-cells in peripheral blood with an indolent clinical course. The disease is rare, and only limited case series have been published. METHODS: We retrospectively studied 11 patients with CLPD-NK diagnosed at our institution between 2005 and 2017. RESULTS: Patients included 7 men and 4 women (median age, 60 years)...
January 31, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29352500/characterization-of-skin-blister-fluids-from-children-with-epstein-barr-virus-associated-lymphoproliferative-disease
#3
Taizo Wada, Tomoko Toma, Hanae Miyazawa, Eiko Koizumi, Tetsujiro Shirahashi, Yusuke Matsuda, Akihiro Yachie
Epstein-Barr virus (EBV)-associated T- or natural killer (NK)-cell lymphoproliferative disease (LPD) is a heterogeneous group of disorders characterized by chronic proliferation of EBV-infected lymphocytes. Patients may present with severe skin manifestations, including hypersensitivity to mosquito bites (HMB) and hydroa vacciniforme (HV)-like eruption, which are characterized by blister formation and necrotic ulceration. Skin biopsy specimens show inflammatory reactions comprising EBV-infected lymphocytes...
January 20, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29242302/a-distinct-subtype-of-epstein-barr-virus-positive-t-nk-cell-lymphoproliferative-disorder-adult-patients-with-chronic-active-epstein-barr-virus-infection-like-features
#4
Keisuke Kawamoto, Hiroaki Miyoshi, Takaharu Suzuki, Yasuji Kozai, Koji Kato, Masaharu Miyahara, Toshiaki Yujiri, Naoki Oishi, Ilseung Choi, Katsumichi Fujimaki, Tsuyoshi Muta, Masaaki Kume, Sayaka Moriguchi, Shinobu Tamura, Takeharu Kato, Hiroyuki Tagawa, Junya Makiyama, Yuji Kanisawa, Yuya Sasaki, Daisuke Kurita, Kyohei Yamada, Joji Shimono, Hirohito Sone, Jun Takizawa, Masao Seto, Hiroshi Kimura, Koichi Ohshima
The characteristics of adult patients with chronic active Epstein-Barr virus infection (adult-onset CAEBV) are poorly recognized, hindering early diagnosis and an improved prognosis. Adult-onset CAEBV (n = 54) diagnosed between 2005 and 2015 were conducted. Adult-onset was defined as an estimated age of onset ≥15 years. To characterize the clinical features of adult-onset CAEBV, we compared them to those of pediatric-onset (estimated age of onset <15 years) patients (n = 75). We compared the prognosis of adult-onset CAEBV with that for patients with nasal-type (n = 37) and non-nasal-type (n = 45) extranodal NK/T-cell lymphoma (ENKTL)...
December 14, 2017: Haematologica
https://www.readbyqxmd.com/read/28954972/steroid-responsive-demyelinating-peripheral-neuropathy-associated-with-chronic-lymphoproliferative-disorders-of-natural-killer-cells
#5
Hironori Sano, Toshihiko Maeda, Masatoshi Omoto, Jun-Ichi Ogasawara, Michiaki Koga, Motoharu Kawai, Takashi Kanda
We herein report the findings of a 67-year-old woman with steroid-responsive multiple mononeuropathy associated with chronic natural killer (NK) cell lymphocytosis. The patient developed progressive, asymmetric weakness and numbness in all four extremities in the course of a three-month period. Nerve conduction studies revealed asymmetric demyelination in both the motor and sensory nerves, and a biopsy specimen of the sural nerve showed a conspicuous difference in the demyelination between the neighboring fascicles and the infiltration of NK cells in the endoneurium...
October 27, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28717070/clinical-features-pathogenesis-and-treatment-of-large-granular-lymphocyte-leukemias
#6
Kazuo Oshimi
Large granular lymphocytes (LGLs) are large lymphocytes with azurophilic granules in their cytoplasm. LGLs are either natural killer (NK) cells or T lymphocytes. Expansions of the LGLs in the peripheral blood are seen in various conditions, including three clonal disorders: T-cell LGL (T-LGL) leukemia, chronic lymphoproliferative disorders of NK cells (CLPD-NK), and aggressive NK-cell leukemia (ANKL). However, the monoclonal and polyclonal expansion of LGLs has been associated with many other conditions. The present article describes these LGL disorders, with special emphasis on the clinical features, pathogenesis, and treatments of the three above-mentioned clonal disorders...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28679993/iatrogenic-immunodeficiency-associated-epstein-barr-virus-ebv-negative-natural-killer-cell-lymphoproliferative-disorder-in-a-patient-undergoing-rheumatoid-arthritis-therapy
#7
Tomoyuki Uchida, Morihiro Inoue, Jian Hua, Shogo Tajima, Yasunori Ota, Masao Hagihara
Here we present a patient with rheumatoid arthritis (RA), who was suspected to have developed malignant lymphoma during immunosuppressive therapy 5 years earlier. She temporarily achieved remission after discontinuing therapy; however, her disease worsened with remittent fever and splenomegaly. Splenic biopsy demonstrated infiltration by abnormal cells, which were positive for CD56 and T cell intracytoplasmic antigen, but negative for CD3 and Epstein-Barr virus (EBV) -encoded RNA. Cytogenetic analysis of bone marrow and lumbar spine tumor revealed common complex karyotype abnormalities...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28431200/the-utility-of-flow-cytometry-in-differentiating-nk-t-cell-lymphoma-from-indolent-and-reactive-nk-cell-proliferations
#8
Sanjay de Mel, Jenny Bei Li, Muhammad Bilal Abid, Tiffany Tang, Hui Ming Tay, Wen Chang Ting, Li Mei Poon, Tae Hoon Chung, Benjamin Mow, Allison Tso, Kiat Hoe Ong, Wee Joo Chng, Te Chih Liu
BACKGROUND: The WHO defines three categories of NK cell malignancies; extra nodal NK/T cell lymphoma (NKTCL), aggressive NK cell leukemia, and the provisional entity chronic lymphoproliferative disorder of NK cells (CLPD-NK). Although the flow cytometric (FC) phenotype of CLPD-NK has been described, studies on FC phenotype of NKTCL are limited. To the best of our knowledge ours is the first study to compare the phenotype of NKTCL, CLPD-NK, reactive NK lymphocytosis (RNKL), and normal NK cells using eight color (8C) FC...
April 21, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/28395105/t-and-nk-cell-lymphomas-and-systemic-lymphoproliferative-disorders-and-the-immunodeficiency-setting-2015-sh-eahp-workshop-report-part-4
#9
Dita Gratzinger, Daphne de Jong, Elaine S Jaffe, Amy Chadburn, John K C Chan, John R Goodlad, Jonathan Said, Yasodha Natkunam
Objectives: The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review immunodeficiency-related T- and natural killer (NK)-cell lymphoproliferations. Methods: The Workshop Panel reviewed 88 T- or NK-cell lymphoproliferations and rendered consensus diagnoses. Results: Hyperplasias of T-cell subsets may be clonal; retained architecture and the clinical setting support a benign diagnosis. Specific associations include hepatosplenic T-cell lymphoma with iatrogenic immunosuppression and breast implants with an indolent variant of anaplastic large cell lymphoma...
February 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28367723/gene-expression-analysis-of-hypersensitivity-to-mosquito-bite-chronic-active-ebv-infection-and-nk-t-lymphoma-leukemia
#10
Kana Washio, Takashi Oka, Lamia Abdalkader, Michiko Muraoka, Akira Shimada, Megumi Oda, Hiaki Sato, Katsuyoshi Takata, Yoshitoyo Kagami, Norio Shimizu, Seiichi Kato, Hiroshi Kimura, Kazunori Nishizaki, Tadashi Yoshino, Hirokazu Tsukahara
The human herpes virus, Epstein-Barr virus (EBV), is a known oncogenic virus and plays important roles in life-threatening T/NK-cell lymphoproliferative disorders (T/NK-cell LPD) such as hypersensitivity to mosquito bite (HMB), chronic active EBV infection (CAEBV), and NK/T-cell lymphoma/leukemia. During the clinical courses of HMB and CAEBV, patients frequently develop malignant lymphomas and the diseases passively progress sequentially. In the present study, gene expression of CD16((-))CD56((+))-, EBV((+)) HMB, CAEBV, NK-lymphoma, and NK-leukemia cell lines, which were established from patients, was analyzed using oligonucleotide microarrays and compared to that of CD56(bright)CD16(dim/-) NK cells from healthy donors...
November 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28056294/-the-understanding-of-epstein-barr-virus-associated-lymphoproliferative-disorder
#11
X G Zhou, Y L Zhang, J L Xie, Y H Huang, Y Y Zheng, W S Li, H Chen, F Liu, H X Pan, P Wei, Z Wang, Y C Hu, K Y Yang, H L Xiao, M J Wu, W H Yin, K Y Mei, G Chen, X C Yan, G Meng, G Xu, J Li, S F Tian, J Zhu, Y Q Song, W J Zhang
In recent years, there are increasing articles concerning Epstein-Barr virus associated lymphoproliferative disorder (EBV+ LPD), and the name of EBV+ LPD is used widely. However, the meaning of EBV+ LPD used is not the same, which triggered confusion of the understanding and obstacles of the communication. In order to solve this problem. Literature was reviewed with combination of our cases to clarify the concept of EBV+ LPD and to expound our understanding about it. In general, it is currently accepted that EBV+ LPD refers to a spectrum of lymphoid tissue diseases with EBV infection, including hyperplasia, borderline lesions, and neoplastic diseases...
December 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28028749/oral-cyclophosphamide-was-effective-for-coombs-negative-autoimmune-hemolytic-anemia-in-cd16-cd56-chronic-lymphoproliferative-disorder-of-nk-cells
#12
Nodoka Sekiguchi, Sayaka Nishina, Toru Kawakami, Hitoshi Sakai, Noriko Senoo, Yasushi Senoo, Toshiro Ito, Hiroshi Saito, Hideyuki Nakazawa, Tomonobu Koizumi, Fumihiro Ishida
An 84-year-old woman was referred to our hospital presenting anemia. Her hemoglobin level was 5.8 g/dL, and white blood cell count was 9400/μL, consisting of 82% lymphocytes. Given the lymphocyte phenotype (CD2+, CD3-, CD16+, and CD56-) and negative whole blood EBV viral load, we made a diagnosis of chronic lymphoproliferative disorder of NK cells (CLPD-NK). We suspected hemolytic anemia because of the high levels of reticulocytes in the peripheral blood and the low haptoglobin value. Although the direct Coombs test was negative and there was no cold agglutination, we examined her red-blood-cell-bound IgG (RBC-IgG), which was elevated...
June 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/27988064/cutaneous-ebv-related-lymphoproliferative-disorders
#13
Alejandro A Gru, Elaine S Jaffe
This article will focus on the cutaneous lymphoproliferative disorders associated with EBV, with an emphasis on the upcoming changes in the revised 4th Edition of the WHO classification of tumors of the hematopoietic system, many of which deal with cutaneous disorders derived from NK-cells or T-cells. Extranodal NK/T-cell lymphoma usually presents in the upper aerodigestive tract, but can involve the skin secondarily. EBV-associated T- and NK-cell lymphoproliferative disorders (LPD) in the pediatric age group include the systemic diseases, chronic active EBV infection (CAEBV) and systemic EBV+ T-cell lymphoma of childhood...
January 2017: Seminars in Diagnostic Pathology
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
#14
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/27408372/cd56-negative-aggressive-t-cell-large-granular-lymphocytic-leukemia
#15
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/27362793/diagnosis-of-chronic-lymphoproliferative-disorders-by-flow-cytometry-using-four-color-combinations-for-immunophenotyping-a-proposal-of-the-brazilian-group-of-flow-cytometry-gbcflux
#16
M M Sales, S I A C P Ferreira, M R V Ikoma, A F Sandes, M P Beltrame, N S Bacal, M C A Silva, M Malvezzi, I G H Lorand-Metze, A Orfao, M Yamamoto
BACKGROUND: Multiparametric flow cytometry (MFC) is a powerful tool for the diagnosis of hematological malignancies and has been useful for the classification of chronic lymphoproliferative disorders (CLPD) according to the WHO criteria. Following the purposes of the Brazilian Group of Flow Cytometry (GBCFLUX), the aim of this report was to standardize the minimum requirements to achieve an accurate diagnosis in CLPDs, considering the different economic possibilities of the laboratories in our country...
June 30, 2016: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/27226916/unusual-indolent-course-of-a-chronic-active-epstein-barr-virus-associated-natural-killer-cell-lymphoproliferative-disorder
#17
Arwa Z Al-Riyami, Khalil Al-Farsi, Murtadha Al-Khabori, Mohammed Al-Huneini, Ibrahim Al-Hadabbi
Natural killer (NK) cell lymphoproliferative disorders are uncommon and the Epstein-Barr virus (EBV) plays an important aetiological role in their pathogenesis. We report a 20-year-old male with a chronic active EBV infection associated with a NK cell lymphoproliferative disorder which had an unusual indolent course. He presented to the Sultan Qaboos University Hospital in Muscat, Oman, in December 2011 with a history of intermittent fever and coughing. Examinations revealed generalised lymphadenopathy, hepatosplenomegaly, leukocytosis, transaminitis, diffuse bilateral lung infiltrates and bone marrow lymphocyte involvement...
May 2016: Sultan Qaboos University Medical Journal
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
#18
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/27047646/t-cell-natural-killer-cell-neoplasms-presenting-as-leukemia-case-series-from-single-tertiary-care-center
#19
Shano Naseem, Maninderbir Kaur, Manupdesh Singh Sachdeva, Jasmina Ahluwalia, Reena Das, Neelam Varma, Subhash Varma
BACKGROUND: Mature T/ NK-cell neoplasms are a rare group of disorders and their presentation as leukemia is even rarer. Most of the previous studies have focused on mature B-cell lineage leukemias and there is a paucity of data on mature T/NK-cell lineage leukemias. We, therefore, planned this study to analyze their spectrum, frequency, morphology and immunophenotypic features. SUBJECTS AND METHODS: All cases of lymphomas presenting as leukemia over a period of two and a half years were evaluated...
January 1, 2016: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/27042473/natural-killer-cell-lymphoma-a-case-with-classification-dilemma
#20
Ankit Kumar Jitani, Yookarin Khonglah, Ritesh Kumar, Bidyut Bikash Gogoi, Ekta Jajodia
Non-Hodgkins lymphoma of the Natural Killer (NK) cell type is rare. World Health Organisation recognises 3 NK-cell phenotypic entities; extranodal NK/T cell lymphoma, nasal type (ENK/TL); aggressive NK cell leukaemia (ANKL); and chronic lymphoproliferative disorders of NK cells (CLPD-NK) which is classified as a provisional entity. Though specific clinical, morphological and immunophenotypic criteria have been laid down to diagnose these conditions there may however, be considerable variations in the clinical presentation making diagnosis difficult...
February 2016: Journal of Clinical and Diagnostic Research: JCDR
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