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Best Practice & Research. Clinical Haematology

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https://www.readbyqxmd.com/read/28288723/clinical-features-and-management-of-non-gastrointestinal-non-ocular-extranodal-mucosa-associated-lymphoid-tissue-enmalt-marginal-zone-lymphomas
#1
REVIEW
Estella Matutes, Carlos Montalban
Extranodal mucosa associated lymphoid tissue (ENMALT) marginal zone lymphomas may arise at any site of the body. The most frequent localizations other than gastrointestinal and eye are salivary gland, skin, lung and thyroid. These lymphomas usually arise in a setting of inflammation due to a persistent infection or autoimmune diseases such as Sjogren syndrome in salivary MALT lymphomas and Hashimoto's thryroiditis in thyroid lymphomas. They affect middle-aged patients with a female predominance when lymphoma arises in certain locations...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288722/nodal-marginal-zone-lymphoma-clinical-features-diagnosis-management-and-treatment
#2
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/28288721/epidemiology-and-environmental-aspects-of-marginal-zone-lymphomas
#3
REVIEW
Priya Sriskandarajah, Claire E Dearden
Marginal zone lymphomas (MZLs) account for between 5% and 17% of all non-Hodgkin's lymphomas. MZLs consist of 3 different subtypes with extranodal being the most commonly reported, representing 50-70% of MZL, followed by splenic (20%) and nodal (10%). Median age at presentation varies between these lymphoma sub-types, ranging between 50 and 69 years, with an overall greater incidence noted in males compared to females. Given the rarity of these lymphomas, epidemiologic data has been sparse, although it has been suggested the aetiology is multi-factorial including ethnicity and geographical factors...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288720/clonal-b-cell-lymphocytosis-of-marginal-zone-origin
#4
REVIEW
Aliki Xochelli, David Oscier, Kostas Stamatopoulos
Monoclonal B cell Lymphocytosis (MBL) is the term used to characterize individuals presenting with lymphocytosis in the absence of lymphadenopathy, organomegaly or any other features suggestive of an active disease. Based on the immunophenotypic findings, MBL cases are sub-categorized into chronic lymphocytic leukemia (CLL)-like, atypical CLL and non-CLL MBL. The latter corresponds to cases with immunophenotypic features suggestive of post germinal center derivation and still represents a diagnostic conundrum...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288719/marginal-zone-lymphoma-associated-autoimmunity-and-auto-immune-disorders
#5
REVIEW
Larissa Sena Teixeira Mendes, Andrew Wotherspoon
Large epidemiological studies have shown a consistent increased risk for developing lymphoma in the setting of autoimmune disorders (AID). It is known that this link appears to be stronger for some AID and certain non-Hodgkin lymphoma subtypes e.g. Sjögren's syndrome and extra-nodal marginal zone lymphoma of the salivary gland, and thyroid MALT lymphoma in a background of Hashimoto's thyroiditis. B and T-cell hyperactivity due to chronic antigenic stimulation and the consequent presence of acquired lymphoid tissue seems to play a key role in the pathogenesis of AI-related lymphomas...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288718/splenic-marginal-zone-lymphoma
#6
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
#7
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
#8
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/28288715/hepatitis-c-virus-associated-marginal-zone-lymphoma
#9
REVIEW
Marine Armand, Caroline Besson, Olivier Hermine, Frédéric Davi
The link between hepatitis C virus (HCV) infection and the development of B-cell non-Hodgkin lymphoma is now well established and based on a number of epidemiological studies. It is further supported by the observation of lymphoma regression after HCV eradication by antiviral treatment. The far most frequent entities are marginal zone lymphoma (MZL) and diffuse large B-cell lymphoma (DLBCL). MZL usually emerge on a background of mixed cryoglobulinemia, a low-grade lymphoproliferation, and often transform into DLBCL, thereby following a multistep oncogenesis process...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288714/bacteria-associated-with-marginal-zone-lymphomas
#10
REVIEW
Maurilio Ponzoni, Andrés J M Ferreri
In the last decades some bacteria have been associated with a various extent with marginal zone (extra nodal, nodal and splenic types) lymphomas 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 Chlamydia psittaci and ocular adnexal MALT-lymphoma and Borrelia burgdorferi and cutaneous MALT lymphoma constitute the most studied examples; in addition, Campylobacter jejuni and some more recent associations encompassing Achromobacter xylosoxidans and Haemophilus influenzae will be further reported...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288713/methylation-patterns-in-marginal-zone-lymphoma
#11
REVIEW
Alberto J Arribas, Francesco Bertoni
Promoter DNA methylation is a major regulator of gene expression and transcription. The identification of methylation changes is important for understanding disease pathogenesis, for identifying prognostic markers and can drive novel therapeutic approaches. In this review we summarize the current knowledge regarding DNA methylation in MALT lymphoma, splenic marginal zone lymphoma, nodal marginal zone lymphoma. Despite important differences in the study design for different publications and the existence of a sole large and genome-wide methylation study for splenic marginal zone lymphoma, it is clear that DNA methylation plays an important role in marginal zone lymphomas, in which it contributes to the inactivation of tumor suppressors but also to the expression of genes sustaining tumor cell survival and proliferation...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288712/the-role-of-stem-cell-transplantation-in-the-treatment-of-marginal-zone-lymphoma
#12
REVIEW
Avichai Shimoni
High-dose chemotherapy and autologous stem-cell transplantation (ASCT) is standard therapy in relapsed/refractory aggressive lymphoma. The optimal therapy of relapsed/refractory disseminated marginal-zone lymphoma (MZL) has not been defined. Limited data on ASCT in this setting suggests outcomes are similar to what is expected in follicular lymphoma. International guidelines suggest that ASCT should be considered in follicular lymphoma in second or subsequent remission, in particular in high-risk disease, or following disease transformation...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288711/marginal-zone-lymphoma-in-elderly-and-geriatric-patients
#13
REVIEW
Valentin Goede
Approximately 50% of patients with newly diagnosed marginal zone lymphoma (MZL) are of advanced age. For the three subtypes of MZL (extranodal MZL of mucosa-associated lymphoid tissue, splenic MZL, nodal MZL), the median age at diagnosis is around 65-70 years. Due to the lack of larger studies in MZL, little is known of the prevalence of comorbidity, polypharmacy, or geriatric syndromes in older patients with MZL. The impact of these concurrent conditions on the tolerability and feasibility of diagnostic or therapeutic procedures used in MZL has not been specifically investigated...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288710/possible-novel-agents-in-marginal-zone-lymphoma
#14
REVIEW
Pier Luigi Zinzani, Alessandro Broccoli
Efficacy, safety and mechanisms of action of novel agents in marginal zone lymphoma patients, both with a nodal and extranodal presentation, are reviewed. Data on lenalidomide, bortezomib and (90)yttrium-ibrutumomab tiuxetan are obtained from trials specifically designed for patients affected by marginal zone lymphoma and with various disease presentations. The role of targeted agents, such as obinutuzumab, ibrutinib and idelalisib, and of some very new drugs (venetoclax, copanlisib, ublituximab and TGR-1202) is also discussed, taking into account the most relevant experiences in patients with indolent non-Hodgkin's lymphomas...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288709/treatment-of-splenic-marginal-zone-lymphoma
#15
REVIEW
Christina Kalpadakis, Gerassimos A Pangalis, Maria K Angelopoulou, Theodoros P Vassilakopoulos
Splenic marginal zone lymphoma (SMZL) is a distinct lymphoma entity characterized by an indolent clinical course and prolonged survival. Treatment is not standardized, since there are no prospective randomized trials in large series of SMZL patients. Splenectomy and rituximab represent the most effective treatment strategies used so far. The addition of chemotherapy to rituximab has not further improved the outcome, although this issue requires further investigation. Rituximab monotherapy has been associated with high response rates (∼90%), with approximately half of these responses being complete, even at the molecular level...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288708/transformation-of-marginal-zone-lymphoma-and-association-with-other-lymphomas
#16
REVIEW
Carla Casulo, Jonathan Friedberg
Marginal zone lymphomas (MZL) are a diverse group of indolent lymphoproliferative disorders that comprise three subtypes: nodal, splenic and mucosal associated marginal zone lymphomas (MALT). Histologic transformation (HT) to an aggressive lymphoma is a rare event that can occur in any subtype, and at lower frequency compared to other indolent non Hodgkin lymphomas (NHL) like follicular lymphoma. There are few data directly associated with risk and prognosis of transformation in MZL. However, recent advances in the understanding of molecular and genetic features of MALT have contributed to an evolving appreciation of HT in this disease...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288707/malt-lymphoma-genetic-abnormalities-immunological-stimulation-and-molecular-mechanism
#17
REVIEW
Ming-Qing Du
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) occurs at diverse anatomic sites and is closely associated with several distinct chronic inflammatory disorders. Both the acquired genetic abnormalities and active chronic immunological responses play a critical role in the development of MALT lymphoma, interestingly by dysregulating similar molecular mechanisms. The three translocations seen in MALT lymphoma, namely t(14;18)(q32;q21)/IGH-MALT1, t(1;14)(p22;q32)/BCL10-IGH, and t(11;18)(q21;q21)/BIRC3 (API2)-MALT1 are capable of activating both canonical and non-canonical NF-κB pathways...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288706/ocular-adnexal-marginal-zone-lymphoma-clinical-presentation-pathogenesis-diagnosis-prognosis-and-treatment
#18
REVIEW
Marianna Sassone, Maurilio Ponzoni, Andrés J M Ferreri
Ocular adnexal marginal zone lymphoma (OAML) represents 1-2% of all non Hodgkin lymphomas. In the last few years many advances in understanding the pathogenesis and the molecular basis involved in its development have been done. Many potential risk factors have been proposed; a dysregulation of immune response in association with a chronic antigenic stimulation, have been hypothesized as possible pathogenic mechanism. In particular, Chlamydia psittaci infection has been related to OAML arising, and eradicating antibiotic therapy has been addressed as a safe and cost-effective approach...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288705/clinical-aspects-and-therapy-of-gastrointestinal-malt-lymphoma
#19
REVIEW
Catherine Thieblemont, Emanuele Zucca
Extranodal marginal zone B-cell lymphomas of the mucosa associated lymphoid tissue (MALT) arise from lymphoid populations that are induced by chronic inflammation in extranodal sites. Among the MALT lymphomas, gastrointestinal (GIT) MALT lymphoma is the most frequent compared to non-GIT MALT lymphoma arising from other sites. Gastric MALT lymphoma has been the first to be described with the evidence of an etiopathogenetic link provided by the association between Helicobacter pylori-positive gastritis and gastric MALT lymphoma...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288704/marginal-zone-lymphomas-reconsidering-similarities-and-differences-while-moving-towards-personalized-treatment
#20
EDITORIAL
Emanuele Zucca, Aaron Polliack, Franco Cavalli
No abstract text is available yet for this article.
March 2017: Best Practice & Research. Clinical Haematology
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