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Hepatosplenic lymphoma

Irappa Madabhavi, Gaurang Modi, Harsha Panchal, Apurva Patel, Swaroop Revannasiddaiah, Asha Anand, Sonia Parikh, Kshitij Joshi, Malay Sarkar
Peripheral T cell lymphomas are a heterogeneous group of post-thymic, mature lymphoid malignancies, accounting for approximately 10-15% of all non-Hodgkin's lymphomas. Hepatosplenic T-cell lymphoma (HSGDTCL) is a rare entity, which is characterized by primary extra nodal disease with typical sinusoidal or sinusal infiltration of the liver and the spleen, respectively by expression of the T-cell receptor γδ chain, and by a number of other frequent clinicopathologic features, including aggressive course of disease...
October 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
Zlatko Djurić, Ljiljana Šaranac, Ivana Budić, Voja Pavlović, Jelena Djordjević
The main role of therapy in Crohn's disease is to achieve long-term clinical remission, and to allow for normal growth and development of children. The immunomodulatory drugs used for the maintenance of remission in Crohn's disease include thiopurines (azathioprine and 6-mercaptopurine) and methotrexate. Development of hepatosplenic T-cell lymphoma in some patients with inflammatory bowel disease, treated with thiopurines independently or in combination with anti-tumor necrosis factor agents, resulted in a growing interest in the therapeutic application of methotrexate in children suffering from Crohn's disease...
January 16, 2018: Bosnian Journal of Basic Medical Sciences
Mariko Yabe, Roberto N Miranda, L Jeffrey Medeiros
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and clinically aggressive type of T-cell lymphoma that arises most often in adolescents and young adults. Patients with HSTCL commonly present with B-symptoms and cytopenias which may suggest a diagnosis of acute leukemia initially. Patients present with extranodal disease involving the spleen, liver and bone marrow; lymphadenopathy is usually absent. The lymphoma cells can show a spectrum of cell sizes and are of T-cell lineage, often negative for CD4 and CD8 and positive for T-cell receptor γδ or, less often, αβ...
January 11, 2018: Human Pathology
Neha Nigam, Chhagan Bihari
No abstract text is available yet for this article.
December 2017: Blood Research
D B Chen, D H Shen, H Zhang, Y Wang, Q J Song, S M Yang, X Z Fang
Objective: To study the clinicopathologic features, diagnosis and differential diagnosis of the tumors of lymphoidand hematopoietic tissue of the spleen(TLTS). Methods: Fifty-three cases of TLTS were selected from the pathologic files from Peking University People's Hospital from April 2002 to April 2017. According to WHO classification of tumors of hematopoietic and lymphoid tissues (2008) and its updated classification (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination...
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Saniya Sharma, Sreejesh Sreedharanunni, Man Updesh Singh Sachdeva, Gaurav Prakash, Neelam Varma
No abstract text is available yet for this article.
July 2017: Indian Journal of Pathology & Microbiology
Nariman Deravi, Olaf Berke, J Paul Woods, Dorothee Bienzle
Canine lymphoma is a heterogeneous disease with many different subtypes. Lymphoma of T cell type in particular is variable in outcome, and includes subtypes with non-progressive, slowly- and rapidly-progressive disease course. Association of immunotype with disease course is incompletely defined. Here, results of flow cytometric immunotyping of 127 canine T cell lymphomas were analyzed in relation to survival and progression free interval. Samples originated from 101 multicentric, 8 mediastinal, 6 cutaneous, 5 hepatosplenic, 5 gastrointestinal and 2 other anatomic subtypes of T cell lymphoma...
September 2017: Veterinary Immunology and Immunopathology
Louise K Mercer, Anne C Regierer, Xavier Mariette, William G Dixon, Eva Baecklund, Karin Hellgren, Lene Dreyer, Merete Lund Hetland, René Cordtz, Kimme Hyrich, Anja Strangfeld, Angela Zink, Helena Canhao, M Victoria Hernandez, Florence Tubach, Jacques-Eric Gottenberg, Jacques Morel, Jakub Zavada, Florenzo Iannone, Johan Askling, Joachim Listing
BACKGROUND: Lymphomas comprise a heterogeneous group of malignant diseases with highly variable prognosis. Rheumatoid arthritis (RA) is associated with a twofold increased risk of both Hodgkin's lymphoma (HL) and non-Hodgkin's lymphoma (NHL). It is unknown whether treatment with biologic disease-modifying antirheumatic drugs (bDMARDs) affect the risk of specific lymphoma subtypes. METHODS: Patients never exposed to (bionaïve) or ever treated with bDMARDs from 12 European biologic registers were followed prospectively for the occurrence of first ever histologically confirmed lymphoma...
December 2017: Annals of the Rheumatic Diseases
Samia Yasmeen, Omer Waqas, Javeria Munir, Faisal Sultan, Abdul Hameed
Mucormycosis is a life threatening fungal infection and remains an important cause of morbidity and mortality in immunocompromised patients after hematopoietic stem cell transplant. We report here a case of hepatosplenic mucormycosis in a patient after autologous stem cell transplant. A young man with anaplastic large cell lymphoma underwent autologous hematopoietic stem cell transplant after achieving complete remission with standard chemotherapy and consolidative radiotherapy. He was found to have incidental hepatosplenic hypodensities on follow up imaging, that were proved to be mucormycosis on histopathology after getting CT-guided biopsy of splenic lesions...
May 2017: Pakistan Journal of Medical Sciences Quarterly
Mohamed A Kharfan-Dabaja, Ambuj Kumar, Ernesto Ayala, Mehdi Hamadani, Peter Reimer, Christian Gisselbrecht, Francesco d'Amore, Esa Jantunen, Takashi Ishida, Ali Bazarbachi, Francine Foss, Ranjana Advani, Timothy S Fenske, Hillard M Lazarus, Jonathan W Friedberg, Mahmoud Aljurf, Lubomir Sokol, Kensei Tobinai, Eric Tse, Linda J Burns, Julio C Chavez, Nishitha M Reddy, Ritsuro Suzuki, Sairah Ahmed, Auayporn Nademanee, Mohamad Mohty, Ajay K Gopal, Michelle A Fanale, Barbara Pro, Alison J Moskowitz, Anna Sureda, Miguel Angel Perales, Paul A Carpenter, Bipin N Savani
Recognizing the significant biological and clinical heterogeneity of mature T cell and natural killer (NK)/T cell lymphomas, the American Society for Blood and Marrow Transplantation invited experts to develop clinical practice recommendations related to the role of autologous hematopoietic cell transplantation (auto-HCT) and allogeneic HCT (allo-HCT) for specific histological subtypes. We used the GRADE methodology to aid in moving from evidence to decision making and ultimately to generating final recommendations...
November 2017: Biology of Blood and Marrow Transplantation
Alexey Hodkoff, Pasha Bentley, Xiangdong Xu
No abstract text is available yet for this article.
June 2, 2017: Leukemia & Lymphoma
J Dubreuil, J Leenhardt, R Noel, G Salles, P J Valette, A Skanjeti
No abstract text is available yet for this article.
November 2017: Revista Española de Medicina Nuclear e Imagen Molecular
S Cingam, S Patel, N Koshy
INTRODUCTION: Hepatosplenic T-cell lymphoma (HSTCL); is an unusual entity first described in 1990 that predominantly affects middle-aged men and is classified by WHO under peripheral T-cell lymphomas. We present a 26-year-old man with HSCTL treated with a non-CHOP regimen. CASE: A 26 year old immigrant from Cameroon without significant past medical history presented with abdominal discomfort that was first noted 1 month prior at which time he was elbowed in abdomen during a basketball game...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
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
Noriaki Yoshida, David M Weinstock
<b/> In this issue of Cancer Discovery , McKinney and colleagues describe the genetics of hepatosplenic T-cell lymphoma, a rare subtype of T-cell lymphoma with unique clinical characteristics. The findings, specifically frequent mutations of STAT5B , PIK3CD , and the histone methyltransferase SETD2 , may help guide translational efforts to target this deadly disease. Cancer Discov; 7(4); 352-3. ©2017 AACR. See related article by McKinney et al., p. 369 .
April 2017: Cancer Discovery
Shuo-Chieh Wu, Jennifer R Chapman, Francisco Vega, Neil Abrahams, Izidore S Lossos, Juan Pablo Alderuccio
No abstract text is available yet for this article.
March 28, 2017: Leukemia & Lymphoma
Jia Li, Sumedha Roy, Young-Mi Kim, Shibo Li, Baojun Zhang, Cassandra Love, Anupama Reddy, Deepthi Rajagopalan, Sandeep Dave, Anna Mae Diehl, Yuan Zhuang
Inhibitor of DNA binding (Id) proteins, including Id1-4, are transcriptional regulators involved in promoting cell proliferation and survival in various cell types. Although upregulation of Id proteins is associated with a broad spectrum of tumors, recent studies have identified that Id3 plays a tumor-suppressor role in the development of Burkitt's lymphoma in humans and hepatosplenic T cell lymphomas in mice. In this article, we report rapid lymphoma development in Id2/Id3 double-knockout mice that is caused by unchecked expansion of invariant NKT (iNKT) cells or a unique subset of innate-like CD1d-independent T cells...
April 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
Shanxiang Zhang, Michael G Bayerl
Splenic γ/δ T-cell proliferation is rare, and correct diagnosis is critical for adequate clinical management. Two splenectomy cases from patients with splenomegaly and cytopenias were studied by morphological evaluation, extensive immunophenotyping, FISH and molecular studies. The clinicopathologic findings were compared with splenic T γ/δ neoplasia, notably hepatosplenic T-cell lymphoma (HSTL) and T-cell large granular lymphocytic leukemia (TLGL) of the variety T γ/δ. The enlarged spleens showed expanded red pulp with markedly increased γ/δ T cells, which share significant to complete overlapping morphology and immunophenotype with the neoplastic γ/δ T cells in HSTL and γ/δ TLGL...
August 2017: Human Pathology
Xiao Han, Wei Zhang, Daobin Zhou, Jing Ruan, Minghui Duan, Tienan Zhu, Jian Li, Huacong Cai, Xinxin Cao, Mingqi Ouyang
Objective To determine the efficacy and prognosis of autologous hematopoietic stem cell transplantation (ASCT) as frontline treatment for peripheral T cell lymphoma (PTCL). Methods Clinical data from 46 PTCL patients who achieved complete (CR) or partial remission (PR) after ASCT from October 1996 to July 2014 were analysed retrospectively. Results Median patient age was 32 (range: 15-68) years. Disease types included PTCL, unspecified type, in 23 patients, anaplastic large cell lymphoma in eight, angioimmunoblastic lymphoma in eight, extranodal NK/T-cell lymphoma in five, and hepatosplenic T-cell lymphoma and enteropathy associated T-cell lymphoma in one each...
February 2017: Journal of International Medical Research
Matthew McKinney, Andrea B Moffitt, Philippe Gaulard, Marion Travert, Laurence De Leval, Alina Nicolae, Mark Raffeld, Elaine S Jaffe, Stefania Pittaluga, Liqiang Xi, Tayla Heavican, Javeed Iqbal, Karim Belhadj, Marie Helene Delfau-Larue, Virginie Fataccioli, Magdalena B Czader, Izidore S Lossos, Jennifer R Chapman-Fredricks, Kristy L Richards, Yuri Fedoriw, Sarah L Ondrejka, Eric D Hsi, Lawrence Low, Dennis Weisenburger, Wing C Chan, Neha Mehta-Shah, Steven Horwitz, Leon Bernal-Mizrachi, Christopher R Flowers, Anne W Beaven, Mayur Parihar, Lucile Baseggio, Marie Parrens, Anne Moreau, Pierre Sujobert, Monika Pilichowska, Andrew M Evens, Amy Chadburn, Rex K H Au-Yeung, Gopesh Srivastava, William W L Choi, John R Goodlad, Igor Aurer, Sandra Basic-Kinda, Randy D Gascoyne, Nicholas S Davis, Guojie Li, Jenny Zhang, Deepthi Rajagopalan, Anupama Reddy, Cassandra Love, Shawn Levy, Yuan Zhuang, Jyotishka Datta, David B Dunson, Sandeep S Davé
Hepatosplenic T-cell lymphoma (HSTL) is a rare and lethal lymphoma; the genetic drivers of this disease are unknown. Through whole-exome sequencing of 68 HSTLs, we define recurrently mutated driver genes and copy-number alterations in the disease. Chromatin-modifying genes, including SETD2, INO80 , and ARID1B , were commonly mutated in HSTL, affecting 62% of cases. HSTLs manifest frequent mutations in STAT5B (31%), STAT3 (9%), and PIK3CD (9%), for which there currently exist potential targeted therapies. In addition, we noted less frequent events in EZH2, KRAS , and TP53 SETD2 was the most frequently silenced gene in HSTL...
April 2017: Cancer Discovery
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