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NK cell chronic lymphoproliferative disorder

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https://www.readbyqxmd.com/read/28954972/steroid-responsive-demyelinating-peripheral-neuropathy-associated-with-chronic-lymphoproliferative-disorders-of-natural-killer-cells
#1
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...
September 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28717070/clinical-features-pathogenesis-and-treatment-of-large-granular-lymphocyte-leukemias
#2
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
#3
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
#4
Sanjay de Mel, Jenny Bei Li, Muhammad Bilal Abid, Tiffany Pooi Ling 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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#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/27047646/t-cell-natural-killer-cell-neoplasms-presenting-as-leukemia-case-series-from-single-tertiary-care-center
#15
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
#16
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
https://www.readbyqxmd.com/read/26992145/mature-t-and-nk-cell-non-hodgkin-lymphoma-in-children-and-young-adolescents
#17
REVIEW
Vinodh Pillai, Michael Tallarico, Michael R Bishop, Megan S Lim
Mature T/Natural killer (NK)-cell neoplasms of children and the young adolescent population exhibit higher prevalence in Central and South American and Asian populations and many are associated with Epstein-Barr virus (EBV). They are represented in large part by extranodal T/NK cell lymphomas- nasal-type or extra nasal-type, chronic lymphoproliferative disorders of T/NK cells or chronic active EBV disease, systemic EBV-positive lymphoproliferative disorders of childhood, hydroa vacciniforme-like lymphoma, hepatosplenic T-cell lymphoma and primary cutaneous gamma/delta T-cell lymphoma among others...
May 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/26913432/-large-granular-lymphocytic-leukemia-and-jak-stat-signaling-pathway-review
#18
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/26844502/epstein-barr-virus-positive-t-nk-cell-lymphoproliferative-disorders-manifested-as-gastrointestinal-perforations-and-skin-lesions-a-case-report
#19
Hai-Juan Xiao, Ji Li, Hong-Mei Song, Zheng-Hong Li, Mei Dong, Xiao-Ge Zhou
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders (LPDs) of childhood is a highly aggressive EBV-positive T/natural killer (NK)-cell LPD, which emerges in the background of chronic active EBV infection (CAEBV) or shortly after primary acute EBV infection. The clinical presentations of CAEBV are varied; patients with atypical manifestations are easily misdiagnosed. We described a 14-year-old boy suffering from digestive disorders and intermittent fever for 1 year and 9 months, whose conditions worsened and skin lesions occurred 2 months before hospitalization...
February 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/26556869/phenotypic-profile-of-expanded-nk-cells-in-chronic-lymphoproliferative-disorders-a-surrogate-marker-for-nk-cell-clonality
#20
Paloma Bárcena, María Jara-Acevedo, María Dolores Tabernero, Antonio López, María Luz Sánchez, Andrés C García-Montero, Noemí Muñoz-García, María Belén Vidriales, Artur Paiva, Quentin Lecrevisse, Margarida Lima, Anton W Langerak, Sebastian Böttcher, Jacques J M van Dongen, Alberto Orfao, Julia Almeida
Currently, the lack of a universal and specific marker of clonality hampers the diagnosis and classification of chronic expansions of natural killer (NK) cells. Here we investigated the utility of flow cytometric detection of aberrant/altered NK-cell phenotypes as a surrogate marker for clonality, in the diagnostic work-up of chronic lymphoproliferative disorders of NK cells (CLPD-NK). For this purpose, a large panel of markers was evaluated by multiparametric flow cytometry on peripheral blood (PB) CD56(low) NK cells from 60 patients, including 23 subjects with predefined clonal (n = 9) and polyclonal (n = 14) CD56(low) NK-cell expansions, and 37 with CLPD-NK of undetermined clonality; also, PB samples from 10 healthy adults were included...
December 15, 2015: Oncotarget
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