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Marginal Zone Lymphoma

V Pillonel, D Juskevicius, C K Y Ng, A Bodmer, A Zettl, D Jucker, S Dirnhofer, A Tzankov
Nodal marginal zone lymphoma (NMZL) is a rare small B-cell lymphoma lacking disease-defining phenotype and precise diagnostic markers. To better understand the mutational landscape of NMZL, particularly in comparison to other nodal small B-cell lymphomas, we performed whole-exome sequencing, targeted high-throughput sequencing, and array-comparative genomic hybridization on a retrospective series. Our study identified for the first time recurrent, diagnostically useful, and potentially therapeutically relevant BRAF mutations in NMZL...
February 28, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Tal Corina Sela, Ofrat Beyar Katz, Tamar Tadmor, Jacob Bejar, Elad Schiff
No abstract text is available yet for this article.
March 2018: Israel Medical Association Journal: IMAJ
Aby Z Philip
Splenic marginal zone lymphoma (SMZL) is a rare indolent B-cell malignancy involving the spleen and bone marrow. Various cytogenetic abnormalities with prognostic value have been identified in SMZL. Complexity of karyotype, 14q aberrations, and TP53 deletions have been found to be poor prognostic indicators. We report an unusual case of SMZL with a complex karyotype including 17p deletion, primarily refractory to 2 chemoimmunotherapy regimens, that responded well to treatment with phosphatidylinositol-3-kinase delta (PI3Kδ) inhibitors idelalisib and rituximab...
March 2018: Journal of the National Comprehensive Cancer Network: JNCCN
Y Wu, L Zhao, Y Chai
Extranodal marginal zone B-cell lymphoma of mucosa-associated lymph tissue (MALT lymphoma) is a rare subtype of marginal zone B-cell lymphoma. Most primary MALT lymphoma occurs in the stomach, while lung is an uncommon site of MALT lymphoma. We herein report a case of MALT lymphoma in a 44-year-old female who underwent a pulmonary lobectomy and received rituximab plus CHOP chemotherapy regimen. The patient had an uneventful postoperative course and a good prognosis without tumor recurrence for 3 years.
March 2018: Nigerian Journal of Clinical Practice
Soumya Surath Panda, Manas Baisakh, Adyakinkar Panda, Hemlata Das
Primary hepatic lymphoma (PHL) is an extremely rare disease and is often misdiagnosed. The optimal therapy is still unclear and the outcomes are uncertain. Among PHLs, a primary hepatic low-grade marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue is still rarer. The present study reports the case of an elderly female diagnosed with PHL (mucosa-associated lymphoid tissue) and treated with single agent rituximab. After 18 months, she had a progressive disease and developed Waldenstorms macroglobulinemia concomitantly...
January 8, 2018: Current Problems in Cancer
Jacqueline Mupas-Uy, Yoshiyuki Kitaguchi, Yasuhiro Takahashi, Emiko Takahashi, Hirohiko Kakizaki
Purpose: To report an atypical case of cystic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of lacrimal gland associated with vision loss. Observations: An 89-year-old woman was presented with a rapidly progressing proptosis, lagophthalmos, and vision loss. Endophthalmitis was also present. Computed tomography scan images showed a hyperdense mass with hypodense cystic areas occupying the superolateral orbit, which displaced the globe antero-inferiorly with optic nerve compression and stretching...
April 2017: American Journal of Ophthalmology Case Reports
Domenico Albano, Raffaele Giubbini, Francesco Bertagna
PURPOSE: The detection rate and the metabolic behavior of 18F-FDG-PET/CT in splenic marginal zone lymphoma (SMZL) are not yet clear. Our aim was to investigate the metabolic behavior of SMZL and whether the tumor stage (acc. Ann Arbor) epidemiological (age, gender), histological (Ki-67 index, plasmacytic differentiation), and morphological (splenic diameter maximum) features might be related to 18F-FDG PET/CT results. METHODS: Fifty-one patients (34 male, 17 female; average age 70 years) with histologically confirmed SMZL who underwent a 18F-FDG PET/CT for initial staging were included: PET/CT images were analyzed visually and semi-quantitatively (SUVmax, lesion-to-liver SUVmax ratio, and lesion-to-blood pool SUVmax ratio)...
March 3, 2018: Abdominal Radiology
Tibor Mezei, Simona Mocan, Alina Ormenisan, Beáta Baróti, Alina Iacob
Salivary gland tumors are relatively rare neoplasms, mostly located in the parotid gland, and few are malignant. Preoperative evaluation of salivary gland tumors includes fine needle aspiration cytology (FNAC). OBJECTIVE: The purpose of this study was to determine the importance of FNAC in the evaluation of rare salivary gland neoplasms. MATERIAL AND METHODS: Four cases of rare salivary gland tumors were included, which were preoperatively assessed by clinical investigation, computed tomography, and FNAC...
2018: Journal of Applied Oral Science: Revista FOB
Katja Maurus, Silke Appenzeller, Sabine Roth, Jochen Kuper, Simone Rost, Svenja Meierjohann, Panagiota Arampatzi, Matthias Goebeler, Andreas Rosenwald, Eva Geissinger, Marion Wobser
Primary cutaneous marginal zone lymphoma (PCMZL) represents an indolent subtype of Non-Hodgkin lymphomas being clinically characterized by slowly growing tumors of the skin, but a very low propensity for systemic dissemination. Up to now, the underlying genetic basis of PCMZL has not been comprehensively elucidated. With the aim to gain deeper insight into the molecular pathogenesis of PCMZL, we performed hybridization-based panel sequencing of 38 cases of well-characterized PCMZL. In 32 out of 38 cases we identified genetic alterations within 39 selected target genes...
February 23, 2018: Journal of Investigative Dermatology
Shuang Ma, Rachel Jug, Shuai Shen, Wan-Lin Zhang, Hong-Tao Xu, Lian-He Yang
RATIONALE: The palatine tonsil is an important component of Waldeyer's ring and a site commonly involved by lymphoma. Interestingly, although it is a site of mucosa-associated lymphoid tissue (MALT), primary MALT lymphoma of the palatine tonsil is rare, especially with prominent plasmacytic differentiation. PATIENT CONCERNS: A 59-year-old woman presented to the hospital with a 1-month history of odynophagia. The patient had no fever or pruritus during this period and she declared no family history of hematolymphoid malignancy...
January 2018: Medicine (Baltimore)
Mariangeles Auat, Chandra Chiappin Cardoso, Iris Mattos Santos-Pirath, Renata Cristina Messores Rudolf-Oliveira, Camila Matiollo, Bárbara Gil Lange, Jessica Pires da Silva, Gisele Cristina Dametto, Mayara Marin Pirolli, Maria Daniela Holthausen Perico Colombo, Maria Claudia Santos-Silva
BACKGROUND: Flow cytometric immunophenotyping is deemed a fundamental tool for the diagnosis of B-cell neoplasms. Currently, the investigation of novel immunophenotypic markers has gained importance, as they can assist in the precise subclassification of B-cell malignancies by flow cytometry. Therefore, the purpose of the present study was to evaluate the expression of CD307a during normal B-cell maturation and in B-cell malignancies as well as to investigate its potential role in the differential diagnosis of these entities...
February 24, 2018: Cytometry. Part B, Clinical Cytometry
Geffen Kleinstern, Mor Averbuch, Rania Abu Seir, Riki Perlman, Dina Ben Yehuda, Ora Paltiel
Although autoimmune diseases (AIDs) are known to predispose to non-Hodgkin lymphoma (NHL), their association with NHL prognosis has rarely been investigated. We examined associations between autoimmunity and B-cell NHL onset by comparing AID history (determined by self-report and medication review and supplemented by chart review where possible) among 435 adult B-NHL patients in Hadassah-Hebrew University Medical Center, diagnosed 2009-2014, and 414 age-and-sex frequency-matched controls. We examined AIDs as a whole, B- and T-cell-mediated AIDs, and autoimmune thyroid diseases...
February 22, 2018: Hematological Oncology
Cesare Mazzaro, Luigino Dal Maso, Luca Quartuccio, Michela Ghersetti, Marco Lenzi, Endri Mauro, Milena Bond, Pietro Casarin, Valter Gattei, Ivo Maria Crosato, Salvatore De Vita, Gabriele Pozzato
OBJECTIVES: To investigate the long-term effects and safety of new direct anti-viral agents (DAAs) in patients with hepatitis C virus (HCV)-related mixed cryoglobulinaemia (MC) without renal involvement. METHODS: The study enrolled 22 consecutive patients, 19 received sofosbuvir-based regimen and three patients received other DAAs, individually tailored according to latest guidelines. As of December 2016, the median length of follow-up was 17 months (range 13-21)...
February 13, 2018: Clinical and Experimental Rheumatology
Christina Kalpadakis, Gerassimos A Pangalis, Maria K Angelopoulou, Sotirios Sachanas, Theodoros P Vassilakopoulos
SMZL is a relatively rare low grade B-cell lymphoma, characterized usually by an indolent clinical behavior. Since there is no prospective randomized trials to establish the best treatment approach, decision on therapeutic management should be based on the available retrospective series. Based on these data, rituximab and splenectomy appear to be the most effective. Splenectomy represented the standard treatment modality until early 2000s. More than 90% of the patients present quick amelioration of splenomegaly related symptoms along with improvement of cytopenias related to hypersplenism...
March 2018: Best Practice & Research. Clinical Haematology
Irene Defrancesco, Luca Arcaini
Extranodal marginal zone B-cell lymphomas (EMZLs) of the mucosa-associated lymphoid tissue (MALT) are indolent lymphomas which can present at any extranodal site. The most frequent localizations (other than stomach) are ocular adnexa, salivary gland, skin, lung and thyroid. Chronic inflammation and antigenic stimulation are a potential risk for the development of MALT lymphomas. While Helicobacter Pylori (HP) is known to be associated with gastric MALT lymphoma and antibiotic therapy is effective in the setting of HP-positive, other microorganisms (such as Chlamydophila Psittaci, Campylobacter Jejiuni, Borrelia Burgdoferi) have been implicated in the pathogenesis of non-gastric MALT lymphomas...
March 2018: Best Practice & Research. Clinical Haematology
Laila König, Juliane Hörner-Rieber, Denise Bernhardt, Adriane Hommertgen, Stefan Rieken, Jürgen Debus, Klaus Herfarth
PURPOSE: Retrospective study of effectiveness, toxicity, and relapse patterns after low-dose radiotherapy (LDRT) in patients with low-grade lymphomas. METHODS: 47 patients (median age 64 years) with 50 lesions were treated with LDRT (2 × 2 Gy). In 60%, LDRT was the primary and curative treatment, in 40% offered as second-line therapy in recurrent disease. Histology included follicular (57%) and marginal zone lymphomas (43%). Patients were followed-up regularly clinically (skin) and with CT or MRI scans...
February 15, 2018: Strahlentherapie und Onkologie: Organ der Deutschen Röntgengesellschaft ... [et Al]
Shuhua Yi, Zengjun Li, Dehui Zou, Wenjie Xiong, Heng Li, Rui Cui, Chengwen Li, Yuting Yan, Wei Liu, Rui Lv, Zhen Yu, Weiwei Chen, Yan Xu, Gang An, Huijun Wang, Kun Ru, Tao Cheng, Jianxiang Wang, Lugui Qiu
B-cell chronic lymphoproliferative disorders (B-CLPD) comprise several entities with indolent clinical manifestations but heterogeneous survival. Cytogenetic aberrations are now the standard prognostic predictors in chronic lymphocytic leukemia (CLL) but have been less investigated in other subtypes of B-CLPD. In this study, we detected cytogenetic aberrations by fluorescence in situ hybridization (FISH) in 875 B-CLPD patients, based on a panel probes locating at 13q14, 11q22, 17p13 and CEP12. We identified del17p acted as the independent adverse cytogenetic predictor for overall survival (OS) in CLL...
January 9, 2018: Oncotarget
Joji Shimono, Hiroaki Miyoshi, Takeharu Kato, Takeshi Sugio, Kohta Miyawaki, Tomohiko Kamimura, Takuto Miyagishima, Tetsuya Eto, Yoshitaka Imaizumi, Koji Kato, Koji Nagafuji, Koichi Akashi, Masao Seto, Takanori Teshima, Koichi Ohshima
Hepatitis C virus (HCV) is a single-stranded RNA virus that not only affects hepatocytes, by B cells as well. It is thought that HCV is involved in the onset of B-cell lymphoma. The clinicopathological characteristics of HCV-positive diffuse large B-cell lymphoma (DLBCL) and HCV-positive splenic marginal zone lymphoma (SMZL) are known, but there has been no report on HCV-positive follicular lymphoma (FL). In this study, the clinicopathological characteristics of HCV-positive FL were examined in 263 patients with FL who were classified into a HCV-positive group with HCV antibody and negative groups without one...
January 5, 2018: Oncotarget
Alexey Glazyrin, Chirag Patel, Lara Kujtan, Sheshadri Madhusudhana
Low-grade follicular lymphomas are genetically characterized by the translocation t(14; 18)(q32;q21) with BCL2 gene rearrangements. Marginal zone lymphomas are often associated with translocations or transcriptional deregulations of the MALT gene. We report 2 cases of lymphomas which harbor both the t(14;18)(q32;q21) translocation and MALT gene upregulation. Patients presented with numerous circulating atypical lymphocytes. Lymph node biopsy in both cases on HE staining demonstrated vague nodularity readily highlighted by CD10, CD23, or BCL6...
February 6, 2018: Acta Haematologica
Bashar M Bata, Jose S Pulido, Sanjay V Patel, Shakila P Khan, Diva Salomao, Thomas G Boyce, Erick D Bothun
No abstract text is available yet for this article.
February 2, 2018: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
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