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hepatosplenic T cell lymphoma

Philip H Brandt, Leena T Rahmat, Syed S Ali
Hepatosplenic gamma-delta T-cell lymphoma with concurrent hemophogocytic lymphohistiocytosis is a rare but well-recognized clinical scenario, associated with a grim prognosis. Clinicians must be aware of this aggressive type of lymphoma so that a prompt diagnosis can be made with timely initiation of systemic therapy and referral for bone marrow transplant.
January 2019: Clinical Case Reports
Lohith Gowda, Francine Foss
Hepatosplenic T-cell lymphoma (HSTL) is a rare variant of extranodal peripheral T-cell lymphomas (PTCL), associated with aggressive disease course and a relentless track record for lethal outcomes. HSTL presents commonly in young men in their third or fourth decade. Of the known causes, immune dysregulation and immunosuppression are the key players in the pathogenesis of HSTL. Clinical manifestation includes hepatosplenomegaly, fevers, and weakness. Bone marrow involvement or organomegaly can cause cytopenias...
2019: Cancer Treatment and Research
Hemani Jain, Dhanlaxmi Shetty, Hasmukh Jain, Manju Sengar, Navin Khattry, P G Subramanian
Hepatosplenic T-cell lymphoma (HSTL) is a rare subtype of peripheral T-cell lymphoma predominantly seen in young males. This disease presents with isolated hepatosplenomegaly and thrombocytopenia with sinusoidal infiltration of liver and sinusal infiltration of spleen. Immunophenotype shows positivity for CD3, CD7, TCRγδ or TCRαβ, CD38 and double negative for CD4, CD8, TdT, CD5, and CD56. Isochromosome 7q with or without trisomy 8 is seen in HSTL. Recently, ring chromosome 7 has also been identified as a new abnormality...
December 2018: Cancer Genetics
Fan Wu, Ying Pan, Huiping Wang, Qianshan Tao, Furun An, Jiakui Zhang, Zhimin Zhai
Hepatosplenic T-cell lymphoma (HSTCL) is a very rare non-Hodgkin lymphoma with an aggressive clinical course and poor prognosis. Patients of this disease usually presented with hepatosplenomegaly, which can be misdiagnosed or delayed. Bone marrow (BM) and peripheral blood (PB) are frequently involved, however, central nervous system (CNS) involvement is less common. Here, we are reporting an unusual case of hepatosplenic γδ T-cell lymphoma in a 64-year-old man with CNS involvement. Flow cytometry immunophenotyping was proved of great diagnostic contribution...
November 30, 2018: Cytometry. Part B, Clinical Cytometry
Kai Sun, Cesar Gentille Sanchez, Sai Ravi Pingali, Swaminathan Iyer
Hepatosplenic γδ T-cell lymphoma (HSTCL) is a rare aggressive peripheral T-cell lymphoma. Prognosis is usually poor with a median survival between 8 and 16 months after traditional chemotherapy. Stem cell transplantation (SCT) is promising and with a more intense induction regimen, has yielded positive results. We report the use of pegylated-asparaginase (PEG-asparaginase) along with a conventional anthracycline-containing regimen in a 51-year-old male who was diagnosed with HSTCL, achieved a complete remission, and subsequently underwent peripheral blood SCT and remained in remission at the time of this case report...
2018: Ecancermedicalscience
Marika Okuni, Kimikazu Yakushijin, Keiichiro Uehara, Hiroya Ichikawa, Hirotaka Suto, Akiko Hashimoto, Yasuhiro Tanaka, Isaku Shinzato, Rina Sakai, Yu Mizutani, Shigeki Nagao, Keiji Kurata, Seiji Kakiuchi, Yoshiharu Miyata, Yumiko Inui, Yasuyuki Saito, Shinichiro Kawamoto, Katsuya Yamamoto, Mitsuhiro Ito, Hiroshi Matsuoka, Hironobu Minami
A 45-year-old woman was diagnosed with hepatosplenic T-cell lymphoma (HSTCL), a rare subtype of peripheral T-cell lymphoma. She received different types of chemotherapy, but disease progression was observed. To reduce the tumor burden before an unrelated bone marrow transplantation, combination chemotherapy consisting of the gemcitabine, carboplatin, and dexamethasone (GCD) was administered as bridging therapy, resulting in a reduction in the number of lymphoma cells. We were then able to perform bone marrow transplantation...
November 19, 2018: Internal Medicine
Hui Pan, Jing Huang, Jun-Nan Li, Li Yang, Jian-Yu Wang, Xin Wang, Lin Liu, Ze-Song Yang, Li Wang
RATIONALE: Hepatosplenic T-cell lymphoma (HSTCL) is a rare but aggressive type of peripheral T-cell lymphoma (PTCL). There is an urgent need for effective treatment due to the poor prognosis of HSTCL. Here, for the 1st time we describe the rare successful case of HSTCL who relapsed after a previous allogeneic stem-cell transplantation (allo-SCT), achieved remission with the second allo-SCT from the same donor. PATIENT CONCERNS: A 24-year-old male, presented with a 2-week history of fever, drenching night sweats and nonquantified weight loss...
November 2018: Medicine (Baltimore)
Anke K Bergmann, Virginie Fataccioli, Giancarlo Castellano, Nadine Martin-Garcia, Laura Pelletier, Ole Ammerpohl, Juri Bergmann, Jaydeep Bhat, Enrique Carrillo-de Santa Pau, José I Martín-Subero, Andrea B Moffitt, Alfonso Valencia, Hans-Heinrich Oberg, Daniela Wesch, Sandrine Jayne, Martin J S Dyer, Dieter Kabelitz, Philippe Gaulard, Reiner Siebert
No abstract text is available yet for this article.
October 18, 2018: Haematologica
Natalia Pin Chuen Zing, Thais Fischer, Jasmine Zain, Massimo Federico, Steven T Rosen
The World Health Organization classification for peripheral T-cell lymphomas (PTCLs) continues to evolve based on genetic and clinical distinctions of each entity. In Part 1, an overview was provided of PTCL not otherwise specified, follicular T-cell lymphoma, angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma (ALCL), and breast implant-associated ALCL. In Part 2, this review is extended to extranodal natural killer (NK)/T-cell lymphoma, enteropathy-associated T-cell lymphoma, indolent T-cell lymphoproliferative disorder of the gastrointestinal tract, adult T-cell leukemia/lymphoma, and hepatosplenic T-cell lymphoma...
August 15, 2018: Oncology (Williston Park, NY)
Maurilio Ponzoni, Elias Campo, Shigeo Nakamura
Intravascular large B-cell lymphoma (IVLBCL) is a rare, clinically aggressive lymphoma entity characterized by an almost exclusive growth of large cells within the lumen of all sized blood vessels. The reasons for this peculiar localization of neoplastic cells are only partially understood. Clinically, in its classical variant, IVLBCL presents with many nonspecific signs and symptoms such as fever of unknown origin and involvement of the central nervous system and skin. Cases, which show disease limited to the skin, following extensive staging workup, are called cutaneous variants and show a better prognosis...
October 11, 2018: Blood
Ding-Bao Chen, Dan-Hua Shen, Shen-Miao Yang, Xin-Zhi Fang
BACKGROUND: To study the effects of splenectomy on treatment and diagnosis of tumours of lymphoid tissue of the spleen. METHODS: Fifty-three cases were reviewed from Peking University People's Hospital from 2002 to 2017. According to WHO classification of tumours of haematopoietic and lymphoid tissues (2008) and classification updated (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination...
July 27, 2018: Pathology, Research and Practice
Sandeep Nemani, Anu Korula, Bhumi Agrawal, M L Kavitha, Marie Therese Manipadam, Elanthenral Sigamani, Biju George, Alok Srivastava, Auro Viswabandya, Vikram Mathews
Background & objectives: Peripheral T cell lymphomas (PTCLs) are a heterogeneous group of non-Hodgkin's lymphomas (NHLs), with universally poor outcome. This study was undertaken to provide data on demographics and outcomes of patients with PTCL who underwent treatment in a single tertiary care centre in southern India. Methods: Retrospective study was done on all patients (age ≥18 yr) diagnosed with PTCL from January 2007 to December 2012. The diagnosis of PTCL was made according to the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues...
May 2018: Indian Journal of Medical Research
Natalia Pin Chuen Zing, Thais Fischer, Jasmine Zain, Massimo Federico, Steven T Rosen
Peripheral T-cell lymphomas (PTCLs) represent a heterogeneous group of diseases, with low incidence and unique epidemiology and pathobiology; they are usually clinically aggressive, with poor outcomes. There have been significant advances in our understanding of the molecular and signaling alterations seen in these malignancies. These observations have led to novel therapeutic strategies that have had a meaningful impact on outcomes. This two-part series highlights the most important aspects of PTCLs and describes current treatment options and investigative opportunities...
July 15, 2018: Oncology (Williston Park, NY)
Sumedha Roy, Yuan Zhuang
A family of transcription factors known as Id proteins, or inhibitor of DNA binding and differentiation, is capable of regulating cell proliferation, survival and differentiation, and is often upregulated in multiple types of tumors. Due to their ability to promote self-renewal, Id proteins have been considered as oncogenes, and potential therapeutic targets in cancer models. On the contrary, certain Id proteins are reported to act as tumor suppressors in the development of Burkitt's lymphoma in humans, and hepatosplenic and innate-like T cell lymphomas in mice...
August 2018: Frontiers of Medicine
Shohei Iijima, Tomohiro Chiba, Keisuke Maruyama, Kuniaki Saito, Keiichi Kobayashi, Yuki Yamagishi, Junji Shibahara, Nobuyuki Takayama, Yoshiaki Shiokawa, Motoo Nagane
BACKGROUND: Brain involvement of hepatosplenic T cell lymphoma (HSTL) has not been reported so far. CASE DESCRIPTION: We observed an extremely rare case of HSTL, which is a rare and aggressive variant of peripheral T cell lymphoma, generally showing predominant infiltration to the liver, spleen, and bone marrow and involving the brain. A 41-year-old Japanese woman presented with dysarthria and numbness of the right hand. Radiologic examination revealed a single 3-cm mass in the left frontal cortex, which was totally removed...
October 2018: World Neurosurgery
M E Joosse, M A Aardoom, P Kemos, D Turner, D C Wilson, S Koletzko, J Martin-de-Carpi, U L Fagerberg, C Spray, C Tzivinikos, M Sladek, R Shaoul, E Roma-Giannikou, J Bronsky, D E Serban, F M Ruemmele, H Garnier-Lengline, G Veres, I Hojsak, K L Kolho, I H Davies, M Aloi, P Lionetti, S Hussey, G Veereman, C P Braegger, E Trindade, A V Wewer, A C Hauer, A C H de Vries, R Sigall Boneh, C Sarbagili Shabat, A Levine, L de Ridder
BACKGROUND: Risk benefit strategies in managing inflammatory bowel diseases (IBD) are dependent upon understanding the risks of uncontrolled inflammation vs those of treatments. Malignancy and mortality in IBD have been associated with disease-related inflammation and immune suppression, but data are limited due to their rare occurrence. AIM: To identify and describe the most common causes of mortality, types of cancer and previous or current therapy among children and young adults with paediatric-onset IBD...
September 2018: Alimentary Pharmacology & Therapeutics
Qing Chen, Lu-Ting Zhu, Xi-Nan Cen, Ze-Yin Liang, Jin-Ping Ou, Li-Hong Wang, Wen-Sheng Wang, Wei Liu, Zhi-Xiang Qiu, Yu-Jun Dong, Mang-Ju Wang, Yu-Hua Sun, Yue Yin, Qian Wang, Han-Yun Ren
OBJECTIVE: To analyze the incidence of bone marrow involvement in patients with different pathological types of lymphoma. METHODS: The results of bone marrow tests including bone marrow aspiration(BMA), flow cytometry detection, bone marrow biopsy(BMB) and 18 F-FDG PET/CT, were analyzed retrospectively in 702 cases of newly diagnosed lymphoma with bone marrow assessment in our hospital from October 2000 to September 2016. If one of the above-mentioned 4 tests showed positive, the lymphoma patient was judged as bone marrow involved...
June 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
Eva C Schwaneck, Regina Renner, Lara Junker, Hermann Einsele, Ottar Gadeholt, Eva Geissinger, Stefan Kleinert, Michael Gernert, Hans-Peter Tony, Marc Schmalzing
OBJECTIVE: Up to one-third of patients with T cell large granular lymphocyte (T-LGL) leukemia display symptoms of rheumatoid arthritis (RA). In Crohn's disease and psoriasis, treatment with tumor necrosis factor (TNF) inhibitors is associated with hepatosplenic γδ T cell lymphoma and with clonal expansion of γδ T cells, respectively. This study was undertaken to determine the prevalence of clonal T-LGL cells in patients with RA and define risk factors for this rare hematologic malignancy...
December 2018: Arthritis & Rheumatology
Akihisa Suwa, Tetsuya Shimoda
Hemophagocytic syndrome (HPS) is a clinicopathological entity characterized by histiocytic proliferation, with marked hemophagocytosis in the reticuloendothelial organs. HPS caused by lymphoma is termed lymphoma-associated hemophagocytic syndrome (LAHS), and there are few reports on canine and feline LAHS. The objective of this study was to examine the clinical, diagnostic, and clinicopathologic features of LAHS in six dogs. The diagnostic criteria of LAHS consisted of lymphoma, bicytopenia or pancytopenia in the blood, and increased hemophagocytosis in the reticuloendothelial organs...
August 22, 2018: Journal of Veterinary Medical Science
Yunjung Choi, So-Yeon Jeon, Wan-Hee Yoo
No abstract text is available yet for this article.
June 22, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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