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Splenic marginal zone B-cell lymphoma

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https://www.readbyqxmd.com/read/28446319/-progress-on-treatment-of-splenic-marginal-zone-lymphoma-review
#1
Ling Wu, Chen Tian, Yi-Zhuo Zhang
Splenic marginal zone lymphoma (SMZL) is an uncommon indolent B-cell non-Hodgkin's lymphoma. The clinical features of SMZL are splenomegaly, lymphocytosis and cytopenia in peripheral blood. Immunopheno-typically, the neoplastic cells are typically positive for CD45, CD20, CD79a, PAX5, IgD, BCL-2. The previous common used treatment is splenectomy and chemotherapy. Nowadays, rituximab-based chemotherapy regimens has improved the curative effect dramaticlly. IFN-α with or without ribavirin can be used to treat the patients with HCV infection...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28393412/early-stages-in-the-ontogeny-of-small-b-cell-lymphomas-genetics-and-microenvironment
#2
P Ghia, B Nadel, B Sander, K Stamatopoulos, F K Stevenson
In this review, we focus on the mechanisms underlying lymphomagenesis in chronic lymphocytic leukaemia, follicular lymphoma, mantle cell lymphoma and splenic marginal zone lymphoma. The cells of origin of these small B-cell lymphomas are distinct, as are the characteristic chromosomal lesions and clinical courses. One shared feature is retention of expression of surface immunoglobulin. Analysis of this critical receptor reveals the point of differentiation reached by the cell of origin. Additionally, the sequence patterns of the immunoglobulin-variable domains can indicate a role for stimulants of the B-cell receptor before, during and after malignant transformation...
April 10, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28362442/prevalence-of-hepatitis-b-and-hepatitis-c-viral-infections-in-various-subtypes-of-b-cell-non-hodgkin-lymphoma-confirmation-of-the-association-with-splenic-marginal-zone-lymphoma
#3
W Xiong, R Lv, H Li, Z Li, H Wang, W Liu, D Zou, L Qiu, S Yi
No abstract text is available yet for this article.
March 31, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28321090/splenic-marginal-zone-lymphoma-complicated-by-cold-agglutinin-disease
#4
Kiyosumi Ochi, Kazuaki Yokoyama, Nobuhiro Ohno, Yasunori Ota, Arinobu Tojo
Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoma accounting for less than 1% of lymphoid neoplasms and is often associated with autoimmune disorders. A 48-year-old woman presented with severe anemia due to steroid-refractory cold agglutinin disease (CAD), and was referred to our hospital for management of progressive systemic illness and high fever. On admission, she showed elevated serum soluble IL-2R and mild splenomegaly. PET/CT revealed FDG accumulation in the spleen and bone. She was pathologically diagnosed as having splenic marginal zone lymphoma by splenectomy and received 8 cycles of rituximab every 2 weeks, resulting in marked improvement of anemia...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28297625/genomics-of-hairy-cell-leukemia
#5
Enrico Tiacci, Valentina Pettirossi, Gianluca Schiavoni, Brunangelo Falini
Hairy cell leukemia (HCL) is a chronic mature B-cell neoplasm with unique clinicopathologic features and an initial exquisite sensitivity to chemotherapy with purine analogs; however, the disease relapses, often repeatedly. The enigmatic pathogenesis of HCL was recently clarified by the discovery of its underlying genetic cause, the BRAF-V600E kinase-activating mutation, which is somatically and clonally present in almost all patients through the entire disease spectrum and clinical course. By aberrantly activating the RAF-MEK-ERK signaling pathway, BRAF-V600E shapes key biologic features of HCL, including its specific expression signature, hairy morphology, and antiapoptotic behavior...
March 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28295729/targeting-of-b-cell-receptor-signalling-in-b-cell-malignancies
#6
M Jerkeman, M Hallek, M Dreyling, C Thieblemont, E Kimby, L Staudt
Pharmacological agents that inhibit enzymes of the B-cell receptor (BCR) pathway are of increasing importance in the treatment of B-cell malignancies. These include inhibitors of Bruton tyrosine kinase (BTK), phosphatidylinositol 3-kinase (PI3K), splenic tyrosine kinase and protein kinase Cβ. Two agents are already approved in the USA and Europe: ibrutinib, a BTK inhibitor, for the treatment of chronic lymphatic leukaemia (CLL), mantle cell lymphoma (MCL) and Waldenström's macroglobulinemia; and idelalisib, a PI3Kδ inhibitor, for the treatment of CLL and follicular lymphoma...
March 14, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28288722/nodal-marginal-zone-lymphoma-clinical-features-diagnosis-management-and-treatment
#7
REVIEW
Tamar Tadmor, Aaron Polliack
Nodular marginal zone lymphoma (NMZL) is a small B-cell lymphoma involving only lymph nodes and is the least common form of MZL constituting about 10% of cases. Patients usually present with advanced disease which must be distinguished from extranodal MZL with lymph node spread. NMZL shares cytological and immunophenotypic features with MALT and splenic MZL, but has a less favorable prognosis than these two categories. It occurs mostly in adults and pediatric cases are rare. Different therapeutic approaches have been used in NMZL, but because of the small patient numbers involved, more definitive treatment is still anticipated...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288718/splenic-marginal-zone-lymphoma
#8
REVIEW
Miguel A Piris, Arantza Onaindía, Manuela Mollejo
Splenic marginal zone lymphoma (SMZL) is an indolent small B-cell lymphoma involving the spleen and bone marrow characterized by a micronodular tumoral infiltration that replaces the preexisting lymphoid follicles and shows marginal zone differentiation as a distinctive finding. SMZL cases are characterized by prominent splenomegaly and bone marrow and peripheral blood infiltration. Cells in peripheral blood show a villous cytology. Bone marrow and peripheral blood characteristic features usually allow a diagnosis of SMZL to be performed...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288717/pathology-of-nodal-marginal-zone-lymphomas
#9
REVIEW
Stefano Pileri, Maurilio Ponzoni
Nodal marginal zone B cell lymphomas (NMZLs) are a rare group of lymphoid disorders part of the spectrum of marginal zone B-cell lymphomas, which encompass splenic marginal one B-cell lymphoma (SMZL) and extra nodal marginal zone of B-cell lymphoma (EMZL), often of MALT-type. Two clinicopathological forms of NMZL are recognized: adult-type and pediatric-type, respectively. NMZLs show overlapping features with other types of MZ, but distinctive features as well. In this review, we will focus on the salient distinguishing features of NMZL mostly under morphological/immunophenotypical/molecular perspectives in views of the recent acquisitions and forthcoming updated 2016 WHO classification of lymphoid malignancies...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288716/molecular-pathogenesis-of-splenic-and-nodal-marginal-zone-lymphoma
#10
REVIEW
Valeria Spina, Davide Rossi
Genomic studies have improved our understanding of the biological basis of splenic (SMZL) and nodal (NMZL) marginal zone lymphoma by providing a comprehensive and unbiased view of the genes/pathways that are deregulated in these diseases. Consistent with the physiological involvement of NOTCH, NF-κB, B-cell receptor and toll-like receptor signaling in mature B-cells differentiation into the marginal zone B-cells, many oncogenic mutations of genes involved in these pathways have been identified in SMZL and NMZL...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28280994/detection-of-myd88-l265p-and-whim-like-cxcr4-mutation-in-patients-with-igm-monoclonal-gammopathy-related-disease
#11
Xin-Xin Cao, Qi Meng, Hao Cai, Tian-Hua He, Cong-Li Zhang, Wei Su, Jian Sun, Yue Li, Wei Xu, Dao-Bin Zhou, Jian Li
A broad spectrum of diseases are associated with IgM monoclonal gammopathy, including Waldenstrom macroglobulinemia (WM), various types of B cell non-Hodgkin's lymphoma (NHL), multiple myeloma (MM), primary amyloidosis (AL), and monoclonal gammopathy of undetermined significance (MGUS); these are called IgM monoclonal gammopathy related diseases (IgM-RD). We investigated MYD88 L265P and WHIM-like CXCR4 mutations in various IgM-RD. Patients with serum immunofixation electrophoresis confirmed IgM monoclonal gammopathy who had enough material for DNA extraction and presented between January 2008 and October 2016 at Peking Union Medical College Hospital were enrolled in this cohort...
March 9, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28230270/a-phase-2-study-of-lenalidomide-rituximab-cyclophosphamide-and-dexamethasone-lr-cd-for-untreated-low-grade-non-hodgkin-lymphoma-requiring-therapy
#12
Allison Rosenthal, Amylou C Dueck, Stephen Ansell, Katherine Gano, Christopher Conley, Grzegorz S Nowakowski, John Camoriano, Jose F Leis, Joseph R Mikhael, A Keith Stewart, David Inwards, David Dingli, Shaji Kumar, Pierre Noel, Morie Gertz, Luis Porrata, Stephen Russell, Joseph Colgan, Rafael Fonseca, Thomas M Habermann, Prashant Kapoor, Francis Buadi, Nelson Leung, Rodger Tiedemann, Thomas E Witzig, Craig Reeder
Patients with indolent non-Hodgkin lymphoma (NHL) have multiple treatment options yet there is no consensus as to the best initial therapy. Lenalidomide, an immunomodulatory agent, has single agent activity in relapsed lymphoma. This trial was conducted to assess feasibility, efficacy, and safety of adding lenalidomide to rituximab, cyclophosphamide, and dexamethasone (LR-CD) in untreated indolent NHL patients requiring therapy. This was a single institution phase II trial. Treatment consisted of IV rituximab 375 mg/m(2) day 1; oral lenalidomide 20 mg days 1-21; cyclophosphamide 250 mg/m(2) days 1, 8, and 15; and dexamethasone 40 mg days 1, 8, 15, and 22 of a 28-day cycle...
May 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28190866/splenic-marginal-zone-lymphoma-uncovered-after-a-10-year-follow-up-as-anemia-of-unknown-cause
#13
Asumi Koyama, Chieko Shiotani, Toshio Kurihara, Toshiki Mushino, Yukiharu Okamoto, Tatsunori Tamaki, Takashi Ozaki, Kouichi Ohshima, Shinobu Tamura
A 75-year-old man was referred to our hospital for evaluation of persistent anemia. Despite repeated diagnostic tests, including bone marrow aspiration, the cause of his anemia remained unknown. On each occasion, computed tomography had revealed neither swollen lymph nodes nor splenomegaly. After a 10-year follow-up period, he was admitted with general fatigue and had developed splenomegaly as well as the anemia. Bone marrow biopsy revealed increased abnormal lymphocytes with short villi that were positive for CD11c, CD19, CD20, and kappa chain, but not for CD5, CD10, CD23, or cyclin D1, according to flow cytometry...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28105889/splenic-marginal-zone-lymphoma-excellent-outcomes-in-64-patients-treated-in-the-rituximab-era
#14
Adam G Starr, Paolo F Caimi, PingFu Fu, Mira R Massoud, Howard Meyerson, Eric D Hsi, David B Mansur, Sheen Cherian, Brenda W Cooper, Marcos J G De Lima, Hillard M Lazarus, Stanton L Gerson, Deepa Jagadeesh, Mitchell R Smith, Robert M Dean, Brad L Pohlman, Brian T Hill, Basem M William
OBJECTIVES AND METHODS: Splenic marginal zone lymphoma (SMZL) is a rare non-Hodgkin lymphoma. We sought to identify prognostic factors and define outcomes in a cohort of 64 patients with SMZL who were treated at two large academic medical centers in North America in the rituximab era. RESULTS: Over a median follow-up of 37.8 (range 6-167.1) months, Kaplan-Meier estimate of median OS was 156.3 months and median PFS was 52.9 months. On univariate analysis, baseline hemoglobin <12 g/dl was associated with inferior OS (p = 0...
January 20, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28087391/clonal-cytogenetic-abnormalities-are-predictor-in-developing-non-hodgkin-lymphomas
#15
Ying Wang, Yi Xiao, Xiangyu Meng, Heng Zhang, Qinlu Li, Fankai Meng, Lifang Huang, Chunrui Li, Jianfeng Zhou
Pathological analysis is the cornerstone for diagnosing malignant lymphoma. Status of cytogenetic abnormalities is frequently left unexamined if no evidence of malignancy is found in pathological analysis. In this study, we presented 3 cases in which clonal cytogenetic abnormalities were detected but morphological alterations of the same tissue did not support malignant non Hodgkin lymphoma at the first lymph node biopsy. Case 1 is a 55-year-old female with lymphadenopathy neoplastic process confirmed by flow cytometry and polymerase chain reaction (PCR)...
January 11, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/28018857/murine-models-of-splenic-marginal-zone-lymphoma-a-role-for-cav1
#16
REVIEW
Chelsey L Patten, Christine E Cutucache
Dozens of murine models of indolent and aggressive B-cell lymphomas have been generated to date. These include those manifesting chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma (DLBCL), as well as xenografts of mantle cell lymphoma (MCL). These models have led to an improved understanding of disease etiology, B-cell biology, immunomodulation, and the importance of the tumor microenvironment. Despite these efforts in CLL, DLBCL, and MCL, considerably little progress toward a model of splenic marginal zone lymphoma (SMZL) has been accomplished...
2016: Frontiers in Oncology
https://www.readbyqxmd.com/read/27867670/lymphoma-heterogeneity-three-different-histological-pictures-and-one-unique-clone
#17
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-in-mice
#18
Veronica S Gil, Govind Bhagat, Louise Howell, Jiyuan Zhang, Chae H Kim, Sven Stengel, Francisco Vega, Arthur Zelent, Kevin Petrie
Histone deacetylase 9 (HDAC9) is expressed in B cells, and its overexpression has been observed in B-lymphoproliferative disorders, including B-cell non-Hodgkin lymphoma (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)...
December 1, 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
#19
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
#20
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
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