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NK cell chronic lymphoproliferative disorder

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https://www.readbyqxmd.com/read/28056294/-the-understanding-of-epstein-barr-virus-associated-lymphoproliferative-disorder
#1
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
#2
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...
December 27, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27988064/cutaneous-ebv-related-lymphoproliferative-disorders
#3
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...
December 7, 2016: 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
#4
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/27408372/cd56-negative-aggressive-t-cell-large-granular-lymphocytic-leukemia
#5
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
#6
M M Sales, Siacp Ferreira, Mrv Ikoma, A F Sandes, M P Beltrame, N S Bacal, McA Silva, M Malvezzi, Igh 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
#7
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
#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/27047646/t-cell-natural-killer-cell-neoplasms-presenting-as-leukemia-case-series-from-single-tertiary-care-center
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/26285106/post-transplant-lymphoproliferative-disorder-ptld-manifesting-in-the-oral-cavity-of-a-13-year-old-liver-transplant-recipient-ltx
#15
Ewa Krasuska-Sławińska, Izabela Minko-Chojnowska, Joanna Pawłowska, Bożenna Dembowska-Bagińska, Maciej Pronicki, Dorota Olczak-Kowalczyk
BACKGROUND Post-transplant lymphoproliferative disorder (PTLD) is a potential complication of solid organ or bone marrow transplants. The main PTLD risk factors are: the Epstein-Barr virus (EBV), transplant type, and use of immunosuppressants. It mainly consists of an uncontrolled growth of lymphocytes in transplant recipients under chronic immunosuppressive therapy. About 85% of PTLDs are EBV-containing B-cell proliferations; 14% are T-cell proliferations, of which only 40% contain EBV; and the remaining 1% is NK-cell or plasmocyte proliferations...
2015: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/26148205/chronic-active-epstein-barr-virus-infection-with-cutaneous-and-sinus-lymphoproliferation-in-a-white-female-patient-with-25%C3%A2-years-follow-up-an-original-case-report
#16
C Picard, S Gouarin, F Comoz, M Barreau, L Verneuil, X Troussard, A Dompmartin
Chronic active Epstein-Barr virus infection (CAEBV) is characterized by chronic infectious mononucleosis-like symptoms associated with very high viral load, as assessed by quantitative polymerase chain reaction. We present an unusual case in a French woman who was followed up over 25 years with cutaneous and sinus lymphoproliferation. This white woman presented with a long history of recurrent cutaneous necrotic papules of the skin, which started during childhood and healed spontaneously with depressed scars...
November 2015: British Journal of Dermatology
https://www.readbyqxmd.com/read/26022711/the-syndrome-of-hemophagocytic-lymphohistiocytosis-in-primary-immunodeficiencies-implications-for-differential-diagnosis-and-pathogenesis
#17
MULTICENTER STUDY
Sebastian Fn Bode, Sandra Ammann, Waleed Al-Herz, Mihaela Bataneant, Christopher C Dvorak, Stephan Gehring, Andrew Gennery, Kimberly C Gilmour, Luis I Gonzalez-Granado, Ute Groß-Wieltsch, Marianne Ifversen, Jenny Lingman-Framme, Susanne Matthes-Martin, Rolf Mesters, Isabelle Meyts, Joris M van Montfrans, Jana Pachlopnik Schmid, Sung-Yun Pai, Pere Soler-Palacin, Uta Schuermann, Volker Schuster, Markus G Seidel, Carsten Speckmann, Polina Stepensky, Karl-Walter Sykora, Bianca Tesi, Thomas Vraetz, Catherine Waruiru, Yenan T Bryceson, Despina Moshous, Kai Lehmberg, Michael B Jordan, Stephan Ehl
Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical and laboratory criteria. Current criteria were created to identify patients with familial hemophagocytic lmyphohistiocytosis in immediate need of immunosuppressive therapy. However, these criteria also identify patients with infection-associated hemophagocytic inflammatory states lacking genetic defects typically predisposing to hemophagocytic lymphohistiocytosis. These patients include those with primary immunodeficiencies, in whom the pathogenesis of the inflammatory syndrome may be distinctive and aggressive immunosuppression is contraindicated...
July 2015: Haematologica
https://www.readbyqxmd.com/read/26013700/aberrant-nk-cell-associated-marker-cd56-and-cd57-expression-in-chronic-lymphocytic-leukemia
#18
Pranav Dorwal, Simmi Mehra, Amit Pande, Dharmendra Jain, G Smeeta, Ritesh Sachdev, Vimarsh Raina
BACKGROUND: Chronic lymphocytic leukemia (CLL) is one of the commonest leukemias that has been reported extensively throughout the literature. The characteristic phenotype includes co-expression of CD5 and CD23, along with dim expression of light chain and CD22/CD79b, with lack of FMC7. The immunophenotypic scoring system given by Matutes has been used to differentiate CLL from non-CLL chronic lymphoproliferative disorders. Various aberrancies have been described in CLL cases, including abnormal (dim or bright) expression of B cell markers and lineage infidel T cell, myelomonocytic, or rarely Natural killer (NK) cells markers...
September 2015: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/25938379/recent-concise-viewpoints-of-chronic-active-epstein-barr-virus-infection
#19
REVIEW
Motohiko Okano
Chronic active Epstein-Barr virus infection (CAEBV) is characterized mainly by prolonged or intermittent fever, lymphadenopathy and hepatosplenomegaly without definite underlying diseases at the diagnosis. Patients with CAEBV also may have various life-threatening conditions including hematological, neurological, pulmonary, cardiac, digestive tract, ocular and/or dermal disorders. Additionally, during the course of illness, they often develop hematological malignancies such as T cell, NK cell or B cell lymphoproliferative disorder (LPD) and/or lymphoma...
2015: Current Pediatric Reviews
https://www.readbyqxmd.com/read/25758948/acute-inflammatory-demyelinating-polyneuropathy-as-a-manifestation-of-chronic-lymphoproliferative-disorder-of-nk-cells
#20
LETTER
Silvia Richelli, Romina Buono, Sergio Ferrari, Gaetano Vattemi, Salvatore Monaco
No abstract text is available yet for this article.
June 2015: Neurological Sciences
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