The Experts below are selected from a list of 34386 Experts worldwide ranked by ideXlab platform
Catherine Thieblemont - One of the best experts on this subject based on the ideXlab platform.
-
Updates in Splenic and Nodal Marginal Zone Lymphoma
2017Co-Authors: Catherine ThieblemontAbstract:This video reviews updates on the treatment and biology of splenic and nodal Marginal Zone lymphoma.
-
Splenic Marginal Zone Lymphoma: Current Knowledge and Future Directions
Oncology (Williston Park N.Y.), 2012Co-Authors: Catherine Thieblemont, Frederic Davi, Maria-elena Noguera, Josette Brière, Francesco Bertoni, Emanuele Zucca, Alexandra Traverse-glehen, Pascale Felman, Françoise Berger, Gilles SallesAbstract:In this article, we review the current knowledge on the biological findings, clinical features, and therapeutic approaches for splenic Marginal Zone lymphoma.
-
Non-MALT Marginal Zone lymphoma.
Current opinion in hematology, 2011Co-Authors: Catherine Thieblemont, Frederic Davi, Maria-elena Noguera, Josette BrièreAbstract:Purpose of review Non-MALT Marginal Zone lymphoma regroups two subtypes of lymphoma, the splenic Marginal Zone lymphoma (SMZL) and the nodal Marginal Zone lymphoma (NMZL). Although they share a common cell of origin from the ‘Marginal Zone’, they display different clinical characteristics, reflecting probable biological variations according to the organ. Recent findings Within the past decade, new data regarding pathogenic mechanisms as well as therapeutic advances have been reported. Summary SMZL and NMZL often present with disseminated disease at diagnosis, with specific clinical presentation, SMZL with predominant enlarged splenomegaly and NMZL with disseminated nodal involvement. Diagnosis may be difficult among the small B-cell lymphomas and criteria for diagnosis have been recently improved. The therapeutic approaches comprise splenectomy for SMZL, and immunochemotherapy for both of SMZL and NMZL, but with no consensus about the best treatment, except when associated with hepatitis C virus. This review addresses the current knowledge on the biological findings, clinical features and therapeutic approaches for the individual SMZLs and NMZLs.
-
Primary cutaneous Marginal Zone lymphoma.
Critical reviews in oncology hematology, 2009Co-Authors: Stéphane Dalle, Luc Thomas, Brigitte Balme, Charles Dumontet, Catherine ThieblemontAbstract:Primary cutaneous Marginal Zone B-cell lymphoma (PCMZL) is included in the group of extranodal Marginal Zone B-cell lymphoma involving mucosal sites. Many evidences suggest that chronic antigen stimulation is a key-player in its pathogenesis. While Helicobacter pylori seems not to be implicated in PCMZL, Borrelia Burgdorferi's role is still matter of debate since the results are discordant between European and North American/Asian countries. However Borrelia subspecies are different between the studied areas and this difference could be a confounding factor. Then ubiquitous candidate antigen is still missing. Beyond these discrepancies the treatment of diffuse PCMZL has been recently improved. If local therapies (surgery, radiation) are the gold standard for localized disease, rituximab can also be considered as an alternative for disseminated or plurifocal PCMZL.
-
Non-MALT Marginal Zone lymphomas.
Annals of Oncology, 2008Co-Authors: Catherine ThieblemontAbstract:Non-MALT Marginal Zone lymphoma regroups two subtypes of lymphoma, the splenic Marginal Zone lymphoma (SMZL) and the nodal Marginal Zone lymphoma (NMZL). Although they share a common cell of origin from the “Marginal Zone” (MZ), they display different clinical characteristics, reflecting probable biological variations according to the organ. Within the past decade, new data regarding pathogenic mechanisms as well as therapeutic advances have been reported. SMZL and NMZL often present with disseminated disease at diagnosis, with specific clinical presentation, SMZL with predominant enlarged splenomegaly and NMZL with disseminated nodal involvement. Diagnosis may be difficult among the small B-cell lymphomas, and criteria for diagnosis have been recently improved. The therapeutic approaches comprise splenectomy for SMZL, and immunochemotherapy for both of SMZL and NMZL, but with no consensus about the best treatment, except when associated with hepatitis C virus. This review addresses the current knowledge on the biological findings, clinical features, and therapeutic approaches for the individual SMZLs and NMZLs.
Steven H Swerdlow - One of the best experts on this subject based on the ideXlab platform.
-
Cutaneous Marginal Zone lymphomas.
Seminars in diagnostic pathology, 2016Co-Authors: Steven H SwerdlowAbstract:Primary cutaneous Marginal Zone lymphoma (CMZL) is one of the major primary B-cell lymphomas of skin. Two types are recognized: a more common class-chain switched CMZL, and a much less common IgM+ CMZL. The extremely indolent course, together with other features distinct from most other MALT lymphomas, has led some to question whether at least the class-switched cases should be considered an overt lymphoma.
-
Marginal Zone lymphomas with plasmacytic differentiation and related disorders.
American journal of clinical pathology, 2011Co-Authors: Thierry Jo Molina, Steven H Swerdlow, Pei Lin, James R. CookAbstract:Marginal Zone lymphomas of all types (nodal, splenic, and extranodal mucosa-associated lymphoid tissue [MALT]) may show plasmacytic differentiation. Distinguishing Marginal Zone lymphomas from other small B-cell lymphomas with plasmacytic differentiation, especially lymphoplasmacytic lymphoma, or from plasma cell neoplasms may be challenging. Marginal Zone lymphomas with plasmacytic differentiation were discussed in 2 sessions of the 2009 Society for Hematopathology/European Association for Haematopathology Workshop. Session 4 focused on nodal Marginal Zone lymphomas, including cases exhibiting classic features and cases displaying atypical phenotypes. The difficulties of classification of cases with increased numbers of large cells were also discussed. Session 5 examined nonnodal Marginal Zone lymphomas and related entities, including splenic Marginal Zone lymphoma, MALT lymphoma, γ heavy chain disease, and cryoglobulin-associated lymphoproliferative disorders. These cases illustrate the importance of clinical data and, in some cases, phenotypic and cytogenetic findings in appropriately applying the 2008 World Health Organization criteria.
-
Defining the borders of splenic Marginal Zone lymphoma: a multiparameter study.
Human pathology, 2009Co-Authors: Scott D. Dufresne, James R. Cook, Raymond E. Felgar, Rachel L. Sargent, Urvashi Surti, Susanne M. Gollin, Ellen D. Mcphail, Steven H SwerdlowAbstract:Classic splenic Marginal Zone lymphomas are CD5-, CD10-, CD23-, CD43-, and usually IgD+ with biphasic white pulp nodules. However, the 2008 World Health Organization classification accepts splenic Marginal Zone lymphomas with monophasic Marginal Zone-like white pulp nodules and recognizes a group of unclassifiable splenic small B-cell lymphomas. To explore the relationship of classic splenic Marginal Zone lymphomas to these other less well-defined splenic lymphomas, a multiparameter study of 47 splenic Marginal Zone lymphomas and unclassifiable splenic small B-cell lymphomas was performed. Seventeen of 31 splenic Marginal Zone lymphomas were biphasic, and 14 were monophasic (90%-100% Marginal Zone-like white pulp nodules). Sixteen cases were unclassifiable splenic small B-cell lymphomas, most lacking a Marginal Zone-type component. There were many clinical similarities between the 3 groups, including similar survivals. Monophasic and unclassifiable cases were less likely to have a typical splenic Marginal Zone lymphoma phenotype (28.6%, 23.1%) compared with biphasic cases (86.7%), usually because of IgD negativity (P < .003). Thirty-four of 42 (81%) cases had cytogenetic abnormalities by fluorescence in situ hybridization; and 17 of 20 (85%), by classical cytogenetics. The most frequent fluorescence in situ hybridization abnormalities among the splenic Marginal Zone lymphomas were del(7)(q31) (26%), +12 (25%), and +3q27 (27%); and among the unclassifiable cases, +12 (50%) and +3q27 (36%). Five of 6 unclassifiable cases with exclusively small non-Marginal Zone-like lymphocytes involving both white and red pulp had +12 compared with 9 of 34 other cases (P < .02). CDK6 (2 cases) and BCL3 (1 case) rearrangements were only seen in the unclassifiable group. These results support including both biphasic and monophasic cases as splenic Marginal Zone lymphomas, but suggest that the lack of a non-Marginal Zone-like population in the monophasic group is associated with some biologic differences. They also demonstrate a relatively large proportion of unclassifiable cases, including a group with frequent +12.
-
pediatric follicular lymphomas Marginal Zone lymphomas and Marginal Zone hyperplasia
American Journal of Clinical Pathology, 2004Co-Authors: Steven H SwerdlowAbstract:Whereas most B-cell lymphomas in children are of diffuse large B-cell or Burkitt type, other B-cell lymphomas, including follicular and Marginal Zone B-cell lymphomas, rarely occur Pediatric follicular lymphomas (FLs) have many features indistinguishable from those seen in adults but demonstrate an increased proportion that are localized, lack bcl-2 protein expression, lack bcl-2 translocations, and are grade 3 but still indolent. They also tend to have large expansile follicles. Although usually at nodal or tonsillar sites, other extranodal involvement also occurs, with FL of the testis well described. Distinction from potentially clonal but reactive follicular hyperplasia is important. Marginal Zone B-cell lymphomas of nodal and extranodal types are other indolent B-cell neoplasms that usually are seen in adults but also occur in children. The differential diagnosis must include the very recently reported lambda light chain class-restricted Marginal Zone hyperplasias of the tonsil or appendix that might closely mimic a lymphoma. Understanding the full spectrum of B-cell neoplasia in children and its unique features is important for diagnostic, therapeutic, and academic purposes.
Manuela Mollejo - One of the best experts on this subject based on the ideXlab platform.
-
Splenic Marginal Zone lymphoma.
Best practice & research. Clinical haematology, 2016Co-Authors: Miguel A. Piris, Arantza Onaindía, Manuela MollejoAbstract: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. Mutational spectrum of SMZL identifies specific findings, such as 7q loss and NOTCH2 and KLF2 mutations, both genes related with Marginal Zone differentiation. There is a striking clinical variability in SMZL cases, dependent of the tumoral load and performance status. Specific molecular markers such as 7q loss, p53 loss/mutation, NOTCH2 and KLF2 mutations have been found to be associated with the clinical variability. Distinction from Monoclonal B-cell lymphocytosis with Marginal Zone phenotype is still an open issue that requires identification of precise and specific thresholds with clinical meaning.
-
whole exome sequencing in splenic Marginal Zone lymphoma reveals mutations in genes involved in Marginal Zone differentiation
Leukemia, 2014Co-Authors: Nerea Martinez, Manuela Mollejo, Carmen Almaraz, Jose P Vaque, Ignacio Varela, Sophia Derdak, Sergi Beltran, Yolanda Camposmartin, Lidia Agueda, Andrea RinaldiAbstract:Splenic Marginal Zone lymphoma (SMZL) is a B-cell neoplasm whose molecular pathogenesis remains fundamentally unexplained, requiring more precise diagnostic markers. Previous molecular studies have revealed 7q loss and mutations of nuclear factor κB (NF-κB), B-cell receptor (BCR) and Notch signalling genes. We performed whole-exome sequencing in a series of SMZL cases. Results confirmed that SMZL is an entity distinct from other low-grade B-cell lymphomas, and identified mutations in multiple genes involved in Marginal Zone development, and others involved in NF-κB, BCR, chromatin remodelling and the cytoskeleton.
-
Splenic Marginal Zone lymphoma: comprehensive analysis of gene expression and miRNA profiling
Modern Pathology, 2013Co-Authors: Alberto J. Arribas, Miguel A. Piris, Cristina Gómez-abad, Margarita Sánchez-beato, Nerea Martinez, Lorena Dilisio, Felipe Casado, Miguel A Cruz, Patrocinio Algara, Manuela MollejoAbstract:Splenic Marginal Zone lymphoma is a small B-cell neoplasm whose molecular pathogenesis is still essentially unknown and whose differentiation from other small B-cell lymphomas is hampered by the lack of specific markers. We have analyzed the gene expression and miRNA profiles of 31 splenic Marginal Zone lymphoma cases. For comparison, 7 spleens with reactive lymphoid hyperplasia, 10 spleens infiltrated by chronic lymphocytic leukemia, 12 spleens with follicular lymphoma, 6 spleens infiltrated by mantle cell lymphoma and 15 lymph nodes infiltrated by nodal Marginal Zone lymphoma were included. The results were validated by qRT–PCR in an independent series including 77 paraffin-embedded splenic Marginal Zone lymphomas. The splenic Marginal Zone lymphoma miRNA signature had deregulated expression of 51 miRNAs. The most highly overexpressed miRNAs were miR-155 , miR-21 , miR-34a , miR-193b and miR-100 , while the most repressed miRNAs were miR-377 , miR-27b , miR-145 , miR-376a and miR-424 . MiRNAs located in 14q32-31 were underexpressed in splenic Marginal Zone lymphoma compared with reactive lymphoid tissues and other B-cell lymphomas. Finally, the gene expression data were integrated with the miRNA profile to identify functional relationships between genes and deregulated miRNAs. Our study reveals miRNAs that are deregulated in splenic Marginal Zone lymphoma and identifies new candidate diagnostic molecules for splenic Marginal Zone lymphoma.
-
Marginal Zone lymphoma.
Seminars in diagnostic pathology, 2011Co-Authors: Miguel A. Piris, Alberto J. Arribas, Manuela MollejoAbstract:The term Marginal Zone lymphoma includes a collection of different diseases with some shared morphologic and pathogenic features, but distinctive clinical presentation, immunophenotype, molecular abnormalities, and treatment recommendations. This review describes the main features of splenic Marginal Zone lymphoma, nodal Marginal Zone lymphoma, and extranodal Marginal Zone lymphoma, mucosa-associated lymphoid tissue type.
J.h.j.m. Van Krieken - One of the best experts on this subject based on the ideXlab platform.
-
Recognizing nodal Marginal Zone lymphoma: recent advances and pitfalls. A systematic review
Haematologica, 2013Co-Authors: M.a.m. Van Den Brand, J.h.j.m. Van KriekenAbstract:The diagnosis of nodal Marginal Zone lymphoma is one of the remaining problem areas in hematopathology. Because no established positive markers exist for this lymphoma, it is frequently a diagnosis of exclusion, making distinction from other low-grade B-cell lymphomas difficult or even impossible. This systematic review summarizes and discusses the current knowledge on nodal Marginal Zone lymphoma, including clinical features, epidemiology and etiology, histology, and cytogenetic and molecular features. In particular, recent advances in diagnostics and pathogenesis are discussed. New immunohistochemical markers have become available that could be used as positive markers for nodal Marginal Zone lymphoma. These markers could be used to ensure more homogeneous study groups in future research. Also, recent gene expression studies and studies describing specific gene mutations have provided clues to the pathogenesis of nodal Marginal Zone lymphoma, suggesting deregulation of the nuclear factor kappa B pathway. Nevertheless, nodal Marginal Zone lymphoma remains an enigmatic entity, requiring further study to define its pathogenesis to allow an accurate diagnosis and tailored treatment. However, recent data indicate that it is not related to splenic or extranodal lymphoma, and that it is also not related to lymphoplasmacytic lymphoma. Thus, even though the diagnosis is not always easy, it is clearly a separate entity.
-
Extranodal Marginal Zone (MALT) lymphoma in common variable immunodeficiency.
The Netherlands journal of medicine, 2006Co-Authors: Ingrid M.e. Desar, J.h.j.m. Van Krieken, Monique Keuter, J.m.m. Raemaekers, Jurjen Jansen, J.w.m. Van Der MeerAbstract:We describe two patients with common variable immunodeficiency (CVID) who developed extranodal Marginal Zone lymphoma (formerly described as mucosa-associated lymphoid tissue lymphoma or MALT lymphoma). One patient, with documented pernicious anaemia and chronic atrophic gastritis with metaplasia, developed a Helicobacter pylori-positive extranodal Marginal Zone lymphoma in the stomach. Three triple regimens of antibiotics were necessary to eliminate the H. pylori, after which the lymphoma completely regressed. Patient B had an H. pylori-negative extranodal Marginal Zone lymphoma of the parotid gland, which remarkably regressed after treatment with clarithromycin. Reviewing the literature, we found eight cases of extranodal Marginal Zone lymphoma complicating CVID, but probably many more cases labelled as non-Hodgkin's lymphoma are hidden in the literature. Until more data are available on the predictive value of noninvasive screening for pathology of the stomach, we recommend endoscopy to assess the gastric status in CVID patients in order to detect these malignancies at an early stage. Elimination of H. pylori infection is the treatment of choice in Helicobacter-positive extranodal Marginal Zone lymphoma. The possibility of elimination failure, most probably due to frequent and prolonged exposure to antibiotics in this patient group, should be taken into account. Treatment with antibiotics in Helicobacter-negative extranodal Marginal Zone lymphoma must be considered.
Maurilio Ponzoni - One of the best experts on this subject based on the ideXlab platform.
-
Pathology of nodal Marginal Zone lymphomas
Best Practice & Research Clinical Haematology, 2016Co-Authors: Stefano Pileri, Maurilio PonzoniAbstract: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.
-
Marginal Zone lymphomas and infectious agents.
Seminars in cancer biology, 2013Co-Authors: Andres J M Ferreri, Silvia Govi, Maurilio PonzoniAbstract:A link with infectious agents, bacteria and viruses in particular, has been reported for many lymphoma entities. Marginal Zone lymphomas (extranodal, nodal and splenic forms) are frequently associated with chronic infections, with important clinical, molecular, biological, and therapeutic implications. The well-known correlation between Helicobacter pylori and gastric MALT-lymphoma, the recently reported links between Chlamydophila psittaci and ocular adnexal MALT-lymphoma and Borrelia burgdorferi and cutaneous MALT lymphoma constitute the best studied examples of lymphomagenic activity of bacteria, while the hepatitis C virus represents the most extensively investigated virus associated with Marginal Zone lymphomas. Biological and clinical features, therapeutic implications and future perspectives of these lymphoma-microbial associations are discussed in this review.
