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Splenic marginal zone B-cell lymphoma

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https://www.readbyqxmd.com/read/27867670/lymphoma-heterogeneity-three-different-histological-pictures-and-one-unique-clone
#1
Sara Alonso-Alvarez, Alba Redondo-Guijo, Óscar Blanco, Miguel Alcoceba, Ana Balanzategui, Juan C Caballero, Julio Dávila, Marcos González, María D Caballero, Alejandro Martín, Ramón García-Sanz
We report a patient who developed up to three different lymphomas with the same clonal IGH rearrangement. She was first diagnosed of splenic zone marginal lymphoma and relapsed for the first time with Hodgkin lymphoma histology and later with diffuse large B-cell lymphoma histology. Subsequent biopsies and analysis of clonally rearranged IGH genes helped to elucidate the clonal relationship between the three histologies and to confirm a common origin from the three tissue histologies. An integrated diagnosis should always be performed in order to achieve the most accurate diagnosis and be able to choose the best therapeutic options for our patients...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27799148/deregulated-expression-of-hdac9-in-b-cells-promotes-development-of-lymphoproliferative-disease-and-lymphoma
#2
V S Gil, G Bhagat, L Howell, J Zhang, C H Kim, S Stengel, F Vega, A Zelent, K Petrie
Histone Deacetylase 9 (HDAC9) is expressed in B-cells and its overexpression has been observed in B-lymphoproliferative disorders and B-cell non-Hodgkin lymphomas (B-NHL). We examined HDAC9 protein expression and copy number alterations in primary B-NHL samples, identifying high HDAC9 expression among various lymphoma entities and HDAC9 copy number gains in 50% of diffuse large B-cell lymphoma (DLBCL). To study the role of HDAC9 in lymphomagenesis we generated a genetically engineered mouse (GEM) model that constitutively expressed an HDAC9 transgene throughout B-cell development under the control of the immunoglobulin heavy chain (IgH) enhancer (Eμ)...
October 28, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/27790140/the-droplet-digital-pcr-a-new-valid-molecular-approach-for-the-assessment-of-b-raf-v600e-mutation-in-hairy-cell-leukemia
#3
Francesca Guerrini, Matteo Paolicchi, Francesco Ghio, Elena Ciabatti, Susanna Grassi, Serena Salehzadeh, Giacomo Ercolano, Maria R Metelli, Marzia Del Re, Lorenzo Iovino, Iacopo Petrini, Giovanni Carulli, Nadia Cecconi, Martina Rousseau, Giulia Cervetti, Sara Galimberti
Hairy cell leukemia (HCL) is a chronic lymphoproliferative B-cell disorder where the B-RAF V600E mutation has been recently detected, as reported for solid neoplasias but not for other B-cell lymphomas. The digital droplet PCR (dd-PCR) is a molecular technique that, without standard references, is able to accurately quantitate DNA mutations. ddPCR could be an useful instrument for the detection of the B-RAF V600E mutation in HCL, where the minimal residual disease monitoring is fundamental for planning a patients-targeted treatment in the era of new anti-CD20 and anti-RAF compounds...
2016: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/27670424/frequent-nfkbie-deletions-are-associated-with-poor-outcome-in-primary-mediastinal-b-cell-lymphoma
#4
Larry Mansouri, Daniel Noerenberg, Emma Young, Elena Mylonas, Maysaa Abdulla, Mareike Frick, Fazila Asmar, Viktor Ljungström, Markus Schneider, Kenichi Yoshida, Aron Skaftason, Tatjana Pandzic, Blanca Gonzalez, Anna Tasidou, Nils Waldhueter, Alfredo Rivas-Delgado, Maria Angelopoulou, Marita Ziepert, Christopher Maximilian Arends, Lucile Couronné, Dido Lenze, Claudia D Baldus, Christian Bastard, Jessica Okosun, Jude Fitzgibbon, Bernd Dörken, Hans G Drexler, Damien Roos-Weil, Clemens A Schmitt, Helga D Munch-Petersen, Thorsten Zenz, Martin-Leo Hansmann, Jonathan C Strefford, Gunilla Enblad, Olivier A Bernard, Elisabeth Ralfkiaer, Martin Erlanson, Penelope Korkolopoulou, Magnus Hultdin, Theodora Papadaki, Kirsten Grønbæk, Armando Lopez-Guillermo, Seishi Ogawa, Ralf Küppers, Kostas Stamatopoulos, Niki Stavroyianni, George Kanellis, Andreas Rosenwald, Elias Campo, Rose-Marie Amini, German Ott, Theodoros P Vassilakopoulos, Michael Hummel, Richard Rosenquist, Frederik Damm
We recently reported a truncating deletion in the NFKBIE gene, which encodes IκBε, a negative feedback regulator of NF-κB, in clinically aggressive chronic lymphocytic leukemia (CLL). Because preliminary data indicate enrichment of NFKBIE aberrations in other lymphoid malignancies, we screened a large patient cohort (n = 1460) diagnosed with different lymphoid neoplasms. While NFKBIE deletions were infrequent in follicular lymphoma, splenic marginal zone lymphoma, and T-cell acute lymphoblastic leukemia (<2%), slightly higher frequencies were seen in diffuse large B-cell lymphoma, mantle cell lymphoma, and primary central nervous system lymphoma (3% to 4%)...
December 8, 2016: Blood
https://www.readbyqxmd.com/read/27666766/immunohistochemical-analysis-of-the-novel-marginal-zone-b-cell-marker-irta1-in-malignant-lymphoma
#5
Jun-Ichiro Ikeda, Masaharu Kohara, Yoko Tsuruta, Satoshi Nojima, Shinichiro Tahara, Kenji Ohshima, Masako Kurashige, Naoki Wada, Eiichi Morii
Marginal zone lymphoma (MZL) is a low-grade B-cell lymphoma derived from marginal-zone B cells. Because of a lack of specific immunohistochemical markers, MZL is mainly diagnosed based on the cytological appearance and growth pattern of the tumor. Marginal-zone B cells were recently shown to selectively express immunoglobulin superfamily receptor translocation-associated 1 (IRTA1), but the antibody used in that study is not commercially available. We therefore investigated the IRTA1 expression in non-neoplastic lymphoid tissues and 261 malignant lymphomas, examining the ability of a commercially available antibody to accurately diagnose MZL...
September 22, 2016: Human Pathology
https://www.readbyqxmd.com/read/27503810/nf-%C3%AE%C2%BAb-deregulation-in-splenic-marginal-zone-lymphoma
#6
Valeria Spina, Davide Rossi
Splenic marginal zone lymphoma is a rare mature B-cell malignancy involving the spleen, bone marrow and blood. Over the past years, the rapid expansion of sequencing technologies allowing the genome-wide assessment of genomic, epigenetic and transcriptional changes has revolutionized our understanding of the biological basis of splenic marginal zone lymphoma by providing a comprehensive and unbiased view of the genes/pathways that are deregulated in this disease. NF-κB is a family of transcription factors that plays critical roles in development, survival, and activation of B lymphocytes...
August 2016: Seminars in Cancer Biology
https://www.readbyqxmd.com/read/27468211/hepatitis-c-virus-associated-b-cell-non-hodgkin-s-lymphoma
#7
REVIEW
Romeo-Gabriel Mihăilă
The hepatitis C virus (HCV) infected patients are prone to develop bone marrow or various tissue infiltrates with monoclonal B cells, monoclonal B lymphocytosis or different types of B cell non-Hodgkin's lymphoma (BCNHL), of which the most common are splenic marginal zone BCNHL, diffuse large BCNHL and follicular lymphoma. The association between chronic HCV infection and non Hodgkin's lymphoma has been observed especially in areas with high prevalence of this viral infection. Outside the limitations of some studies that have been conducted, there are also geographic, environmental, and genetic factors that contribute to the epidemiological differences...
July 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27413523/bendamustine-and-rituximab-as-first-line-treatment-in-intermediate-high-risk-splenic-marginal-zone-lymphomas-of-elderly-patients
#8
Roberto Castelli, Antonio Gidaro, Giorgio Lambertenghi Deliliers
BACKGROUND: Splenic marginal zone lymphoma (SMZL) is a chronic B-cell lymphoproliferative disorder, comprising less than 2% of non-Hodgkin's lymphomas, and affecting mainly middle-aged and elderly patients with a median survival of >10 years. The typical clinical features of SMZL include splenomegaly. Treatment should be patient-tailored and can range from a 'watchful waiting' approach for asymptomatic patients without cytopenias to surgery, localized radiation therapy or immuno/chemotherapies...
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27338637/a-study-of-the-mutational-landscape-of-pediatric-type-follicular-lymphoma-and-pediatric-nodal-marginal-zone-lymphoma
#9
Michael G Ozawa, Aparna Bhaduri, Karen M Chisholm, Steven A Baker, Lisa Ma, James L Zehnder, Sandra Luna-Fineman, Michael P Link, Jason D Merker, Daniel A Arber, Robert S Ohgami
Pediatric-type follicular lymphoma and pediatric marginal zone lymphoma are two of the rarest B-cell lymphomas. These lymphomas occur predominantly in the pediatric population and show features distinct from their more common counterparts in adults: adult-type follicular lymphoma and adult-type nodal marginal zone lymphoma. Here we report a detailed whole-exome deep sequencing analysis of a cohort of pediatric-type follicular lymphomas and pediatric marginal zone lymphomas. This analysis revealed a recurrent somatic variant encoding p...
October 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27335277/the-genetics-of-nodal-marginal-zone-lymphoma
#10
Valeria Spina, Hossein Khiabanian, Monica Messina, Sara Monti, Luciano Cascione, Alessio Bruscaggin, Elisa Spaccarotella, Antony B Holmes, Luca Arcaini, Marco Lucioni, Fabrizio Tabbò, Sakellarios Zairis, Fary Diop, Michaela Cerri, Sabina Chiaretti, Roberto Marasca, Maurilio Ponzoni, Silvia Deaglio, Antonio Ramponi, Enrico Tiacci, Laura Pasqualucci, Marco Paulli, Brunangelo Falini, Giorgio Inghirami, Francesco Bertoni, Robin Foà, Raul Rabadan, Gianluca Gaidano, Davide Rossi
Nodal marginal zone lymphoma (NMZL) is a rare, indolent B-cell tumor that is distinguished from splenic marginal zone lymphoma (SMZL) by the different pattern of dissemination. NMZL still lacks distinct markers and remains orphan of specific cancer gene lesions. By combining whole-exome sequencing, targeted sequencing of tumor-related genes, whole-transcriptome sequencing, and high-resolution single nucleotide polymorphism array analysis, we aimed at disclosing the pathways that are molecularly deregulated in NMZL and we compare the molecular profile of NMZL with that of SMZL...
September 8, 2016: Blood
https://www.readbyqxmd.com/read/27297662/homeobox-nkx2-3-promotes-marginal-zone-lymphomagenesis-by-activating-b-cell-receptor-signalling-and-shaping-lymphocyte-dynamics
#11
Eloy F Robles, Maria Mena-Varas, Laura Barrio, Sara V Merino-Cortes, Péter Balogh, Ming-Qing Du, Takashi Akasaka, Anton Parker, Sergio Roa, Carlos Panizo, Idoia Martin-Guerrero, Reiner Siebert, Victor Segura, Xabier Agirre, Laura Macri-Pellizeri, Beatriz Aldaz, Amaia Vilas-Zornoza, Shaowei Zhang, Sarah Moody, Maria Jose Calasanz, Thomas Tousseyn, Cyril Broccardo, Pierre Brousset, Elena Campos-Sanchez, Cesar Cobaleda, Isidro Sanchez-Garcia, Jose Luis Fernandez-Luna, Ricardo Garcia-Muñoz, Esther Pena, Beatriz Bellosillo, Antonio Salar, Maria Joao Baptista, Jesús Maria Hernandez-Rivas, Marcos Gonzalez, Maria Jose Terol, Joan Climent, Antonio Ferrandez, Xavier Sagaert, Ari M Melnick, Felipe Prosper, David G Oscier, Yolanda R Carrasco, Martin J S Dyer, Jose A Martinez-Climent
NKX2 homeobox family proteins have a role in cancer development. Here we show that NKX2-3 is overexpressed in tumour cells from a subset of patients with marginal-zone lymphomas, but not with other B-cell malignancies. While Nkx2-3-deficient mice exhibit the absence of marginal-zone B cells, transgenic mice with expression of NKX2-3 in B cells show marginal-zone expansion that leads to the development of tumours, faithfully recapitulating the principal clinical and biological features of human marginal-zone lymphomas...
June 14, 2016: Nature Communications
https://www.readbyqxmd.com/read/27241147/-pathologic-characteristics-of-bone-marrow-for-%C3%A2-cd5-positive-small-b-cell-lymphoma
#12
Shusong Peng, Fuqiang Zhu, Sijing You, Minna Gong, Zhonglian Wen
OBJECTIVE: To study the pathologic characteristics of bone marrow for CD5 positive small B cell lymphoma (SBL).
 METHODS: The pathologic profiles of 92 patients with CD5 positive SBL were retrospectively analyzed. The morphologic and immunophenotypic features were analyzed by flow cytometry and immunohistochemistry. IgH/CCND1 was examined by fluorescence in situ hybridization (FISH).
 RESULTS: A total of 92 patients with CD5 positive SBL were enrolled in this study, including 56 (60...
April 2016: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/27131623/a-single-center-experience-in-splenic-diffuse-red-pulp-lymphoma-diagnosis
#13
REVIEW
Hunan L Julhakyan, L S Al-Radi, T N Moiseeva, K I Danishyan, A M Kovrigina, S M Glebova, S A Lugovskaya, V N Dvirnik, A N Khvastunova, I A Yakutik, V G Savchenko
The World Health Organization 2008 classification highlighted a new nosology-splenic diffuse red pulp lymphoma (SDRPL) with clinical and laboratory features similar to both splenic marginal zone lymphoma and hairy cell leukemia (HCL) and variant form of HCL. Experience of hematologists on the diagnosis and differential diagnosis of SDRPL is extremely limited. The aim of our report was to characterize the clinical and immunomorphologic features of SDRPL on our own observations. During 2013-2014, in National Research Center for Hematology, 87 spleen specimens removed from various B-cell lymphomas were analyzed...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/27121112/genetic-aberrations-in-small-b-cell-lymphomas-and-leukemias-molecular-pathology-clinical-relevance-and-therapeutic-targets
#14
Agata M Bogusz, Adam Bagg
Small B-cell lymphomas and leukemias (SBCLs) are a clinically, morphologically, immunophenotypically and genetically heterogeneous group of clonal lymphoid neoplasms, including entities such as chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), mantle cell lymphoma (MCL), follicular lymphoma (FL), lymphoplasmacytic lymphoma (LPL), marginal zone lymphoma (MZL) and hairy cell leukemia (HCL). The pathogenesis of some of these lymphoid malignancies is characterized by distinct translocations, for example t(11;14) in the majority of cases of MCL and t(14;18) in most cases of FL, whereas other entities are associated with a variety of recurrent but nonspecific numeric chromosomal abnormalities, as exemplified by del(13q14), del(11q22), and +12 in CLL, and yet others such as LPL and HCL that lack recurrent or specific cytogenetic aberrations...
September 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27030189/-visceral-leishmaniasis-concurrent-with-splenic-marginal-zone-b-cell-lymphoma
#15
U L Julhakyan, A U Magomedova, V N Dvirnyk, S K Kravchenko
Splenic marginal zone B-cell lymphoma (SMZBCL) is a rare non-Hodgkin B-cell lymphoma that presents with morphologically mature lymphoid cells corresponding in their immunological characteristics to secondary follicular marginal zone lymphocytes. It is clinically characterized by splenomegaly, moderate lymphocytosis, usually focal bone marrow lesion, sometimes moderate of monoclonal immunoglobulin in the serum (generally IgM or IgG) and/or urea, and a relatively benign course. Leishmaniasis is a transmissible natural focal infectious endemic disease that has a great diversity of clinical manifestations...
2016: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/26989207/splenic-marginal-zone-lymphoma-from-genetics-to-management
#16
REVIEW
Luca Arcaini, Davide Rossi, Marco Paulli
Splenic marginal zone lymphoma (SMZL) is a rare B-cell malignancy involving the spleen, bone marrow, and frequently the blood. SMZL lymphomagenesis involves antigen and/or superantigen stimulation and molecular deregulation of genes (NOTCH2 and KLF2) involved in the physiological differentiation of spleen marginal zone B cells. Diagnosis requires either spleen histology or, alternatively, the documentation of a typical cell morphology and immunophenotype on blood cells coupled with the detection of intrasinusoidal infiltration by CD20(+) cells in the bone marrow...
April 28, 2016: Blood
https://www.readbyqxmd.com/read/26945339/incidental-and-isolated-follicular-lymphoma-in-situ-and-mantle-cell-lymphoma-in-situ-lack-clinical-significance
#17
Glenda Bermudez, Sonia González de Villambrosía, Azahara Martínez-López, Ana Batlle, José B Revert-Arce, Laura Cereceda Company, César Ortega Bezanilla, Miguel A Piris, Santiago Montes-Moreno
Follicular lymphoma in situ (FLIS) and mantle cell lymphoma in situ (MCLIS) are histopathologic findings of undetermined clinical significance. We studied a series of 341 consecutive lymph node resection specimens from patients diagnosed with colorectal (201 cases) and breast (140 cases) adenocarcinoma between 1998 and 2000. Incidental and isolated FLIS was identified in 11/341 patients (3.23%), whereas incidental and isolated MCLIS was found in 2/341 patients (0.59%). None of these cases developed overt lymphoma...
July 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/26858354/identification-of-a-novel-stereotypic-ighv4-59-ighj5-encoded-b-cell-receptor-subset-expressed-by-various-b-cell-lymphomas-with-high-affinity-rheumatoid-factor-activity
#18
LETTER
Richard J Bende, Jerry Janssen, Thera A M Wormhoudt, Koen Wagner, Jeroen E J Guikema, Carel J M van Noesel
No abstract text is available yet for this article.
May 2016: Haematologica
https://www.readbyqxmd.com/read/26815349/-expression-of-lats-mrna-in-mantle-cell-lymphoma-and-its-clinical-significance
#19
Zhen Yu, Shuhua Yi, Yanru Zhang, Zengjun Li, Lugui Qiu
OBJECTIVE: To investigate the expression level of large tumor suppressor (LATS)1, LATS2 mRNA and its prognostic value in mantle cell lymphoma (MCL). METHODS: A total of 36 B-NHL cases (including MCL 16 cases, chronic lymphoblastic leukemia (CLL) 11 cases, splenic marginal zone cell lymphoma (SMZL) 9 cases) and 8 healthy donors were enrolled in this study from January 2008 to April 2011 in our Lymphoma Clinic Center. The mRNA level of Yap (effector of Hippo pathway) and LATS1, LATS2 mRNA were detected by using real-time quantitative PCR...
October 2015: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/26795750/acquired-c1-esterase-inhibitor-deficiency-in-lymphomas-prevalence-symptoms-and-response-to-treatment
#20
Christine Bekos, Thomas Perkmann, Maria Krauth, Markus Raderer, Klaus Lechner, Ulrich Jaeger
We retrospectively studied the prevalence of C1 esterase inhibitor (C1 INH) deficiency in 131 patients with various lymphomas. We determined C1 INH activity, C1 INH antigen, and C4 concentration at diagnosis and after chemotherapy. In follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL) and chronic lymphocytic leukemia (CLL) consecutive patients were studied. In these entities, the prevalence of C1 INH deficiency was 10.2% in DLBCL, 4.1% in CLL, and 0% in FL and Hodgkin lymphoma. In indolent lymphomas, we identified only single cases of C1 INH deficiency, predominantly in splenic marginal zone lymphomas (SMZL) (four cases)...
September 2016: Leukemia & Lymphoma
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