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Marginal Zone Lymphoma

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https://www.readbyqxmd.com/read/28329553/primary-cutaneous-marginal-zone-lymphoma
#1
Joshua Farhadian, Vtaly Terushkin, Shane A Meehan, Jo-Ann Latkowski
Primary cutaneous B cell lymphomas (PCBCL) are thesecond most common type of primary cutaneouslymphoma. The three main types of PCBCL areprimary cutaneous marginal-zone lymphoma(PCMZL), primary cutaneous follicle-centerlymphoma, and primary cutaneous diffuse largeB-cell lymphoma, leg type. PCMZL has an indolentcourse with a five-year survival rate approaching99%. Lesions most often present on the trunk or armas erythematous-to-violaceous papules, plaques, ornodules. Approximately one-half of patients havesolitary skin lesions...
December 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28327924/long-term-outcome-of-487-patients-with-early-stage-extra-nodal-marginal-zone-lymphoma
#2
S Teckie, S Qi, M Chelius, S Lovie, M Hsu, A Noy, C Portlock, J Yahalom
Background: Localized early-stage extra-nodal marginal zone lymphoma (MZL) presents with heterogeneous organ involvement and is treated with various modalities, including resection, radiotherapy, and systemic therapy. We report the long-term outcome of a large cohort of extra-nodal MZL and assess the impact of patient and disease characteristics, organ site, and treatment strategy on disease control and survival. Patients and Methods: We identified 487 consecutive patients with stage IE or IIE MZL referred between 1992 and 2012 to Memorial Sloan Kettering Cancer Center...
January 23, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28321090/splenic-marginal-zone-lymphoma-complicated-by-cold-agglutinin-disease
#3
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/28317310/pediatric-nodal-marginal-zone-lymphoma-in-a-japanese-boy
#4
Daiki Hori, Ryoji Kobayashi, Daisuke Suzuki, Hirozumi Sano, Kunihiko Kobayashi
No abstract text is available yet for this article.
March 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28316228/-extranodal-marginal-zone-lymphoma-of-mucosa-associated-lymphoid-tissue-in-the-brain-parenchyma-a-case-report
#5
F Chen, X L Liu, P W Li, X Y Yang, D W Chen
No abstract text is available yet for this article.
March 23, 2017: Zhonghua Zhong Liu za Zhi [Chinese Journal of Oncology]
https://www.readbyqxmd.com/read/28297625/genomics-of-hairy-cell-leukemia
#6
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
#7
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/28294400/chronic-amastigote-negative-cutaneous-leishmaniasis-a-clinical-histopathologic-and-molecular-study-of-27-cases-with-emphasis-on-atypical-and-pseudolymphomatous-presentations
#8
Carlo Tomasini, Laura Moneghini, Maria Barbui Anna
BACKGROUND: Chronic amastigote-negative cutaneous leishmaniasis (CL) is a diagnostic challenge, as the parasite load may be low, or absent in biopsy tissue sections. METHODS: A series of consecutive biopsy specimens, taken from 130 patients with a diagnosis of granulomatous dermatitis of unknown etiology, were reviewed. PCR was carried out for Leishmania-specific DNA. RESULTS: A total of 27/130 samples were positive for Leishmania-specific DNA...
March 10, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28289662/mucosa-associated-lymphoid-tissue-lymphoma-of-the-cecum-and-rectum-a-case-report
#9
Myung Jin Nam, Byung Chang Kim, Sung Chan Park, Chang Won Hong, Kyung Su Han, Dae Kyung Sohn, Weon Seo Park, Hee Jin Chang, Jae Hwan Oh
A colonic mucosa-associated lymphoid-tissue (MALT) lymphoma is relatively rare compared to lymphomas of the stomach or small intestine. We present a case of a MALT lymphoma in the cecum and rectum found during screening colonoscopy. A 54-year-old female, who had undergone right-breast-conserving surgery with axillary dissection due to an invasive ductal carcinoma and a left-breast excisional biopsy due to microcalcification following adjuvant chemoradiation therapy 3 years earlier, was found to have 3-mm-sized smooth elevated lesions in both the cecum and rectum...
February 2017: Annals of Coloproctology
https://www.readbyqxmd.com/read/28288723/clinical-features-and-management-of-non-gastrointestinal-non-ocular-extranodal-mucosa-associated-lymphoid-tissue-enmalt-marginal-zone-lymphomas
#10
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
#11
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
#12
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/28288719/marginal-zone-lymphoma-associated-autoimmunity-and-auto-immune-disorders
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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