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Hepatosplenic lymphoma

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https://www.readbyqxmd.com/read/28258199/id2-collaborates-with-id3-to-suppress-invariant-nkt-and-innate-like-tumors
#1
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...
March 3, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28235627/florid-splenic-gamma-delta-t-cell-proliferation-in-patients-with-splenomegaly-and-cytopenias-a-high-stakes-diagnostic-challenge
#2
Shanxiang Zhang, Michael G Bayerl
OBJECTIVES: Splenic gamma/delta T-cell proliferation is rare and correct diagnosis is critical for adequate clinical management. METHODS: 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 gamma/delta neoplasia, notably hepatosplenic T-cell lymphoma (HSTL) and T-cell large granular lymphocytic leukemia (TLGL) of the variety T gamma/delta...
February 21, 2017: Human Pathology
https://www.readbyqxmd.com/read/28222648/autologous-stem-cell-transplantation-as-frontline-strategy-for-peripheral-t-cell-lymphoma-a-single-centre-experience
#3
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
https://www.readbyqxmd.com/read/28122867/the-genetic-basis-of-hepatosplenic-t-cell-lymphoma
#4
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 TP53SETD2 was the most frequently silenced gene in HSTL...
January 25, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28058028/hepatosplenic-t-cell-lymphoma-in-a-47-year-old-crohn-s-disease-patient-on-thiopurine-monotherapy
#5
Maartje M van de Meeberg, Lauranne A A P Derikx, Harm A M Sinnige, Peet Nooijen, D Lucette Schipper, Loes H C Nissen
Hepatosplenic T-cell lymphoma (HSTCL) is a rare non-Hodgkin lymphoma with a high mortality rate. Higher incidence is reported in patients with inflammatory bowel disease, specifically in male patients that are younger than 35 years, and have been treated with thiopurine and tumor necrosis factor (TNF)-α inhibitor combination therapy for over 2 years. In this case report we describe a 47-year-old patient with Crohn's disease (CD) who developed HSTCL after having been treated with thiopurine monotherapy for 14 years...
December 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28038706/hepatosplenic-t-cell-lymphoma-arising-in-patients-with-immunodysregulatory-disorders-a-study-of-7-patients-who-did-not-receive-tumor-necrosis-factor-%C3%AE-inhibitor-therapy-and-literature-review
#6
Mariko Yabe, L Jeffrey Medeiros, Yahya Daneshbod, Masoud Davanlou, Carlos E Bueso-Ramos, Elisa J Moran, Ken H Young, Roberto N Miranda
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that can arise in patients with underlying immune disorders. Others have suggested that tumor necrosis factor (TNF)-α inhibitor therapy for immune disorders increases the risk of HSTCL. To assess for a potential relationship between HSTCL and the use of TNF-α inhibitors, we searched for patients with HSTCL and underlying immune disorders at our institution. We identified 7 patients with a median age of 38 years. Five patients had Crohn disease, 1 ulcerative colitis, and 1 rheumatoid arthritis...
February 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/27843927/alopecia-areata-as-a-manifestation-of-systemic-lymphoma-report-of-two-cases
#7
Yuval Ramot, Alexander Gural, Abraham Zlotogorski
Alopecia areata is a common autoimmune disorder leading to hair loss. It usually affects individuals under the age of 40, and first appearance in older subjects is considered uncommon. Here, we report 2 cases of rapidly progressing alopecia areata, which appeared for the first time in adults. Patient 1 had alopecia universalis, which preceded the identification of hepatosplenic T-cell lymphoma, a rare form of lymphoma. Patient 2 suffered from the ophiasis type of alopecia areata, presenting for the first time following chemotherapy for non-Hodgkin B-cell lymphoma...
September 2016: Skin Appendage Disorders
https://www.readbyqxmd.com/read/27811192/hepatosplenic-t-cell-lymphoma-with-blastoid-morphology-in-a-patient-with-crohn-disease
#8
Jadee L Neff, Dong Chen
No abstract text is available yet for this article.
November 3, 2016: Blood
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
#9
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...
January 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27562705/cutaneous-presentation-of-hepatosplenic-t-cell-lymphoma-a-potential-mimicker-of-primary-cutaneous-gamma-delta-t-cell-lymphoma
#10
Arti Karpate, Carmen Barcena, Daniel Hohl, Bettina Bisig, Laurence de Leval
No abstract text is available yet for this article.
November 2016: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/27516526/two-cases-of-hepatosplenic-t-cell-lymphoma-in-adolescents-treated-for-autoimmune-hepatitis
#11
Florian Brinkert, Philomena Arrenberg, Till Krech, Enke Grabhorn, Ansgar Lohse, Christoph Schramm
We report the first 2 cases of hepatosplenic T-cell lymphoma in adolescents diagnosed with autoimmune hepatitis under long-term treatment with azathioprine. Patients presented with fatigue, hepatosplenomegaly, and pancytopenia. The diagnosis could be confirmed performing biopsies of liver and spleen or bone marrow, which demonstrated infiltration of neoplastic T lymphocytes with the typical phenotype with both markers, CD56(+) and TCRγδ(+) Thus, these cases emphasize the need to constantly reevaluate the administered dose and duration of thiopurines for autoimmune hepatitis, especially in adolescents...
September 2016: Pediatrics
https://www.readbyqxmd.com/read/27469326/cyclin-d1-expression-in-peripheral-t-cell-lymphomas
#12
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...
November 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27408381/a-case-presenting-with-splenic-infarct-diagnosed-as-primary-bone-marrow-cd5-positive-dlbcl-a-clinicopathological-correlation
#13
Anupriya Bansal, Suchi Mittal, Jasmita Dass, Nitin Gupta, P K Agarwal, Jyoti Kotwal
De novo CD5+ Diffuse large B cell lymphoma (DLBCL) is a rare and aggressive subtype of DLBCL. It is a distinct clinicopathologic entity with complex molecular profile and poor prognosis. A 59 year old female presented with pyrexia of unknown origin since 1 month. On examination, there was severe pallor, hepatosplenomegaly and no palpable lymphadenopathy. Complete blood count revealed bicytopenia with normal total leucocyte count. Liver and renal function tests were normal. Ultrasonography abdomen revealed splenic enlargement with two focal lesions attributed to either splenic abscess or infarcts...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27270301/non-anaplastic-peripheral-t-cell-lymphoma-in-children-and-adolescents-an-international-review-of-143-cases
#14
MULTICENTER STUDY
K Mellgren, A Attarbaschi, O Abla, S Alexander, S Bomken, E Bubanska, A Chiang, M Csóka, A Fedorova, E Kabickova, L Kapuscinska-Kemblowska, R Kobayashi, Z Krenova, F Meyer-Wentrup, N Miakova, M Pillon, G Plat, A Uyttebroeck, D Williams, G Wróbel, U Kontny
Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL)...
August 2016: Annals of Hematology
https://www.readbyqxmd.com/read/27244960/hepatic-lymphoma-and-splenic-aspergillosis-mimicking-hepatosplenic-abscesses-from-melioidosis-in-thailand
#15
Kittiyod Poovorawan, Aung Myint Thu, Maleerat Sutherat, Weerapong Phumratanaprapin, Naruemon Wisedopas, Viravarn Luvira, Wirongrong Chierakul
We report here a case of hepatic lymphoma and splenic aspergillosis in an elderly patient with diabetes mellitus, exhibiting hepatosplenic abscesses mimicking melioidosis. Immunohistochemistry confirmed the diagnosis of a diffuse hepatic large B-cell lymphoma. Biopsy of the spleen revealed a clump of fungus with a slender shape and dichotomous branching, morphologically consistent with aspergillosis. Hepatosplenic abscesses are a common presentation in melioidosis, but this case reveals this assumption can lead to misdiagnosis...
March 2016: Southeast Asian Journal of Tropical Medicine and Public Health
https://www.readbyqxmd.com/read/27212158/image-guided-percutaneous-liver-biopsy-in-hepatosplenic-gamma-delta-t-cell-lymphoma-a-single-centre-experience
#16
C-C Wang, L-Y Chen, K Liu, X-Q Liu, D-J Li
OBJECTIVE: This study was to determine whether Image-Guided Percutaneous Liver Biopsy (IGPLB) is a safe and accurate procedure in patients with Hepatosplenic Gamma-Delta T-Cell Lymphoma (HSTCL) characterized by hepatosplenomegaly, and provide a rapid and safe diagnostic approach in this rare group of patients with a generally poor outcome. PATIENTS AND METHODS: We report five patients who underwent IGPLB for an initial diagnosis of HSTCL, in whom diagnosis by bone marrow biopsy and blood smear had failed...
May 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27158719/hepatosplenic-t-cell-lymphoma-an-acute-leukemia-presentation
#17
Isabelle Arnoux, Marie Loosveld
No abstract text is available yet for this article.
January 14, 2016: Blood
https://www.readbyqxmd.com/read/27047646/t-cell-natural-killer-cell-neoplasms-presenting-as-leukemia-case-series-from-single-tertiary-care-center
#18
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/27033291/hepatosplenic-t-cell-lymphoma-in-a-young-immunocompetent-man
#19
Maja Petrova, Manuel Mendes Gomes, Jose Pedro Nascimento Carda, José Pereira de Moura
Hepatosplenic T-cell lymphoma (HSTCL) is a rare, aggressive type of peripheral T-cell lymphoma that is characterised by extranodal disease, with infiltration and proliferation of malignant T-cells within the liver, spleen and bone marrow. The authors report the case of a young immunocompetent man, who was admitted to the hospital with a history of prolonged, unexplained fever, fatigue and weight loss. Initial blood work showed mild pancytopaenia and imaging studies revealed hepatosplenomegaly. The diagnosis was challenging, initially mimicking infectious disease, and it required an extensive investigation that ultimately revealed the characteristic clinical, histopathological and cytogenetic features of HSTCL...
March 31, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/26997444/dyspoietic-changes-associated-with-hepatosplenic-t-cell-lymphoma-are-not-a-manifestation-of-a-myelodysplastic-syndrome-analysis-of-25-patients
#20
Mariko Yabe, L Jeffrey Medeiros, Guilin Tang, Sa A Wang, Keyur P Patel, Mark Routbort, Govind Bhagat, Carlos E Bueso-Ramos, Jeffrey L Jorgensen, Rajyalakshmi Luthra, Weina Chen, Tariq Muzzafar, Rashmi Kanagal-Shamanna, Joseph D Khoury, Yahya Daneshbod, Masoud Davanlou, Shaoying Li, Ken H Young, Roberto N Miranda
Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell lymphoma commonly associated with cytopenias. The pathogenesis of cytopenias in patients with HSTCL is not well defined, although the presence of dyspoietic hematopoietic cells and the common association with trisomy 8 raise the possibility of an associated myelodysplastic syndrome (MDS). In 25 bone marrow specimens involved by HSTCL, we systematically assessed for morphologic features of dyspoiesis and correlated the findings with peripheral cytopenia(s), cytogenetic findings, and detection of chromosome 8 by fluorescence in situ hybridization...
April 2016: Human Pathology
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