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Nodal marginal zone B-cell lymphoma

Joseph Hatem, April M Schrank-Hacker, Christopher D Watt, Jennifer J D Morrissette, Adam I Rubin, Ellen J Kim, Sunita D Nasta, Mariusz A Wasik, Agata M Bogusz
BACKGROUND: Diffuse large B-cell lymphoma (DLBCL) typically leads to effacement of the nodal architecture by an infiltrate of malignant cells. Rarely (<1%), DLBCL can present with an interfollicular pattern (DLBCL-IF) preserving the lymphoid follicles. It has been postulated that DLBCL-IF is derived from marginal zone B cells and may represent a large-cell transformation of marginal zone lymphoma (MZL), however no direct evidence has been provided to date. Here we describe a rare case of a diagnostically challenging DLBCL-IF involving a lymph node in a patient with a prior history of lymphadenopathy for several years and MZL involving skin...
December 19, 2016: Diagnostic Pathology
Jan P Nicolay, Marion Wobser
Cutaneous B-cell lymphomas (CBCLs) comprise a group of mature lymphoproliferative B-cell disorders that primarily affect the skin. Characterized by great biological and clinical variability among its various subtypes, CBCLs fundamentally differ from primary nodal or systemic B-cell lymphomas. Given their uncomplicated course and excellent prognosis, lymphoma classifications rank primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous follicle center lymphoma (PCFCL) as indolent CBCLs. By contrast, diffuse large B-cell lymphoma, leg type (DLBCL-LT) in particular, represent more aggressive lymphoma variants associated with a poorer prognosis...
December 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Jun-Ichiro Ikeda, Masaharu Kohara, Yoko Tsuruta, Satoshi Nojima, Shinichiro Tahara, Kenji Ohshima, Masako Kurashige, Naoki Wada, Eiichi Morii
Marginal zone lymphoma (MZL) is a low-grade B-cell lymphoma derived from marginal zone B cells. Because of a lack of specific immunohistochemical markers, MZL is mainly diagnosed based on the cytological appearance and growth pattern of the tumor. Marginal zone B cells were recently shown to selectively express immunoglobulin superfamily receptor translocation-associated 1 (IRTA1), but the antibody used in that study is not commercially available. We therefore investigated the IRTA1 expression in nonneoplastic lymphoid tissues and 261 malignant lymphomas, examining the ability of a commercially available antibody to accurately diagnose MZL...
January 2017: Human Pathology
Bikram Shrestha, Bernard Kim, Alison Huffstetler
MALT: Mucosa-associated lymphoid tissue (MALT)-type lymphoma is a relatively rare disease; nevertheless, it is the third most common lymphoma type, accounting for 5-7% of all non-Hodgkin lymphomas. Case series and retrospective analysis published in the literature have suggested that extra gastrointestinal (GI) MALT-type lymphoma can occur simultaneously with MALT-type lymphoma involving the GI tract. We report the case of a healthy, 64-year-old Caucasian male who presented with progressive fatigue, non-productive cough, and worsening exertional shortness of breath for 3 months who was subsequently diagnosed with gastric extra-nodal marginal zone B-cell lymphoma or MALToma with simultaneous metastasis to the lung (bronchi) based on biopsy reports...
2016: Journal of Community Hospital Internal Medicine Perspectives
Kamel Laribi, Adrien Tempescul, Habib Ghnaya, Nathalie Denizon, Anne Besançon, Andreea Anghel, Jonathan Farhi, Catherine Truong, Pierre Lemaire, Stephanie Poulain, Delphine Bolle, Jean Christophe Ianotto, Alix Baugier de Materre
Primary nodal marginal zone lymphoma (NMZL) is a rare disease. There is no current consensus on how to treat it. The bendamustine plus rituximab (BR) regimen is effective for the treatment of follicular and other indolent lymphomas, but its efficacy in NMZL is not known. We analyzed the outcome of 14 patients diagnosed with NMZL (median age 67 years) who were treated with 375 mg/m(2) of rituximab on day 1 and 90 mg/m(2) of bendamustine on days 1 and 2. The overall and complete response rates were 93% and 71%, respectively...
July 22, 2016: Hematological Oncology
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
Nodal marginal zone lymphoma (NMZL) is a rare non-Hodgkin lymphoma that arises from mature B-cells. We delineate outcomes, prognostic factors and treatment trends among a large cohort of patients with NMZL in the rituximab era. We identified 56 such patients treated at our institutions. The majority presented with advanced stage disease (78·6%). Over a median follow-up of 38·2 months, median progression-free survival (PFS) was 42·4 months and median overall survival (OS) was not reached. Kaplan-Meier estimates of OS at 120 months after diagnosis was 71·9%...
October 2016: British Journal of Haematology
Michael G Ozawa, Aparna Bhaduri, Karen M Chisholm, Steven A Baker, Lisa Ma, James L Zehnder, Sandra Luna-Fineman, Michael P Link, Jason D Merker, Daniel A Arber, Robert S Ohgami
Pediatric-type follicular lymphoma and pediatric marginal zone lymphoma are two of the rarest B-cell lymphomas. These lymphomas occur predominantly in the pediatric population and show features distinct from their more common counterparts in adults: adult-type follicular lymphoma and adult-type nodal marginal zone lymphoma. Here we report a detailed whole-exome deep sequencing analysis of a cohort of pediatric-type follicular lymphomas and pediatric marginal zone lymphomas. This analysis revealed a recurrent somatic variant encoding p...
October 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Valeria Spina, Hossein Khiabanian, Monica Messina, Sara Monti, Luciano Cascione, Alessio Bruscaggin, Elisa Spaccarotella, Antony B Holmes, Luca Arcaini, Marco Lucioni, Fabrizio Tabbò, Sakellarios Zairis, Fary Diop, Michaela Cerri, Sabina Chiaretti, Roberto Marasca, Maurilio Ponzoni, Silvia Deaglio, Antonio Ramponi, Enrico Tiacci, Laura Pasqualucci, Marco Paulli, Brunangelo Falini, Giorgio Inghirami, Francesco Bertoni, Robin Foà, Raul Rabadan, Gianluca Gaidano, Davide Rossi
Nodal marginal zone lymphoma (NMZL) is a rare, indolent B-cell tumor that is distinguished from splenic marginal zone lymphoma (SMZL) by the different pattern of dissemination. NMZL still lacks distinct markers and remains orphan of specific cancer gene lesions. By combining whole-exome sequencing, targeted sequencing of tumor-related genes, whole-transcriptome sequencing, and high-resolution single nucleotide polymorphism array analysis, we aimed at disclosing the pathways that are molecularly deregulated in NMZL and we compare the molecular profile of NMZL with that of SMZL...
September 8, 2016: Blood
Masashi Miyaoka, Tomoki Kikuchi, Joaquim Carreras, Yukie Yara Kikuti, Ken Omachi, Minoru Kojima, Kiyoshi Ando, Naoya Nakamura
Composite CD10-positive low-grade B-cell and CD5-positive low-grade B-cell lymphoma is extremely rare. We report a case of a composite follicular lymphoma (FL) and CD5-positive nodal marginal zone lymphoma (NMZL) in a resected inguinal lymph node of a 72-year-old Japanese male. Histologically, multiple follicles had reactive-germinal centers with tingible body macrophages, a thin mantle zone and a wide marginal zone. The wide marginal zone consisted of medium-sized cells having slightly indented nuclei and clear cytoplasm, indicating monocytoid cells with CD5-positive B-cells...
2016: Journal of Clinical and Experimental Hematopathology: JCEH
Michiel van den Brand, Jos Rijntjes, Konnie M Hebeda, Laura Menting, Carolyn V Bregitha, Wendy B C Stevens, Walter J F M van der Velden, Bastiaan B J Tops, J Han J M van Krieken, Patricia J T A Groenen
AIMS: To investigate the spectrum of mutations in 20 genes involved in B-cell receptor and/or Toll-like receptor signalling resulting in activation of nuclear factor-κB (NF-κB) in 20 nodal marginal zone lymphomas (NMZLs), 20 follicular lymphomas (FLs), and 11 cases of B-cell lymphoma, unclassifiable (BCL-u). METHODS AND RESULTS: Nodal marginal zone lymphomas were diagnosed according to strict criteria, including the expression of at least one putative marginal zone marker (MNDA and/or IRTA1)...
January 2017: Histopathology
Suman B Koganti, Alejandra Lozada, Ernesto Curras, Ajay Shah
INTRODUCTION: Primary breast lymphoma by definition is localized to one or both the breasts with or without involvement of regional lymph nodes with no other extra mammary site involvement. Role of surgery in the treatment armamentarium is less well defined. PRESENTATION OF CASE: A 62year old post-menopausal lady was found to have 1.2×1.2×0.6cm mass in the left breast on diagnostic mammography. Core needle biopsy reveals marginal zone lymphoma. Complete staging work-up was performed which was negative for regional spread and metastatic foci...
2016: International Journal of Surgery Case Reports
Andi K Cani, Moaaz Soliman, Daniel H Hovelson, Chia-Jen Liu, Andrew S McDaniel, Michaela J Haller, Jarred V Bratley, Samantha E Rahrig, Qiang Li, César A Briceño, Scott A Tomlins, Rajesh C Rao
Non-Hodgkin lymphoma of the orbit and ocular adnexa is the most common primary orbital malignancy. Treatments for low- (extra-nodal marginal zone and follicular lymphomas) and high-grade (diffuse large B-cell lymphoma) are associated with local and vision-threatening toxicities. High-grade lymphomas relapse frequently and exhibit poor survival rates. Despite advances in genomic profiling and precision medicine, orbital and ocular adnexal lymphomas remain poorly characterized molecularly. We performed targeted next-generation sequencing (NGS) profiling of 38 formalin-fixed, paraffin-embedded orbital and ocular adnexal lymphomas obtained from a single-center using a panel targeting near-term, clinically relevant genes...
July 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Catherine Thieblemont, Thierry Molina, Frédéric Davi
Nodal marginal zone lymphoma (NMZL) is a rare form of indolent small B-cell lymphoma which has only been clearly identified in the last 2 decades and which to date remains incurable. Progress in therapeutic management has been slow, largely due to the very small number of patients treated and the heterogeneity of treatments administered; thus, standard-of-care treatment is currently nonspecific for this lymphoma entity. In this review, treatments routinely used to manage adult NMZL patients are presented, principally based on immunochemotherapy (when treatment is needed)...
April 28, 2016: Blood
L X Li, S T Zhou, X Ji, H Ren, Y L Sun, J B Zhang, H B Wang, Z W Liu
BACKGROUND: Extra-nodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue originating in the liver is less common. CASE PRESENTATION: We described the clinical presentation, immunohistochemistry, and immunophenotypes of this lymphoma, which was misdiagnosed with tiny hepatic carcinoma in a 44-year-old woman with hepatitis C; the patient underwent left lateral sectionectomy. The immunophenotype identified the most of the lymphoid cells as positive CD20, CD34, Ki67, CD3, CD4, CD79a, CD45RO, MUM-1, and CD5 and negative CD10, CD15, CD30, ACT, CK, CRO, DES, and HMB45...
March 8, 2016: World Journal of Surgical Oncology
Michiel van den Brand, Olga Balagué, Patricia H J van Cleef, Patricia J T A Groenen, Konnie M Hebeda, Daphne de Jong, J Han J M van Krieken
In our consultation practice, it was noted that many cases that were considered to represent follicular lymphoma (FL) without a BCL2 translocation were ultimately classified as nodal marginal zone lymphoma (NMZL). This study set out to define recurrent morphological features of these cases. Thirty-three low-grade B cell lymphomas without a BCL2 rearrangement were studied for recurrent morphological features. These features were then applied on 20 randomly selected cases to verify if these criteria are able to distinguish between lymphomas with and without a BCL2 rearrangement, assigning them to one of five categories ranging from "certain FL" to "certain NMZL...
March 2016: Journal of Hematopathology
Anupam Varshney, Tarang Goyal, Vijay Zawar, Marianne Tinguely, Werner Kempf
Patients with a lymphoma have an increased risk of developing a second lymphoproliferative disorder. The association of nodal Hodgkin lymphoma and primary cutaneous marginal zone lymphoma (MALT type) is exceptional, and only very few cases have been documented. Anetoderma represents a circumscribed loss or rarefication of elastic fibers. Different underlying processes may result in anetoderma, including cutaneous marginal zone lymphoma. We report a 50-year-old male patient with Epstein-Barr virus (EBV)-associated nodal Hodgkin lymphoma who presented with disseminated anetodermic skin lesions...
July 2016: International Journal of Dermatology
Glenda Bermudez, Sonia González de Villambrosía, Azahara Martínez-López, Ana Batlle, José B Revert-Arce, Laura Cereceda Company, César Ortega Bezanilla, Miguel A Piris, Santiago Montes-Moreno
Follicular lymphoma in situ (FLIS) and mantle cell lymphoma in situ (MCLIS) are histopathologic findings of undetermined clinical significance. We studied a series of 341 consecutive lymph node resection specimens from patients diagnosed with colorectal (201 cases) and breast (140 cases) adenocarcinoma between 1998 and 2000. Incidental and isolated FLIS was identified in 11/341 patients (3.23%), whereas incidental and isolated MCLIS was found in 2/341 patients (0.59%). None of these cases developed overt lymphoma...
July 2016: American Journal of Surgical Pathology
Kamel Laribi, Habib Ghnaya, Pierre-Jean Mention, Hoel Rousset, Alix Baugier de Materre, Fabienne Pineau-Vincent, Catherine Truong, Delphine Bolle
A patient with a marginal zone lymphoma received RCHOP and obtained PR. He received RDHAP, autograft, and obtained CR. Three months later, he developed Kaposi's sarcoma with spontaneous regression. Two months later, he developed DLBCL treated with R-MIV with CR. Thereafter, he developed AML and died a few days later.
January 2016: Clinical Case Reports
Salvatore Perrone, Gianna Maria D'Elia, Giorgia Annechini, Alessandro Pulsoni
Marginal zone lymphomas have been associated with several infectious agents covering both viral and bacterial pathogens and in some cases a clear aetiological role has been established. Pathogenetic mechanisms are currently not completely understood. However, the role of chronic stimulation of the host immune response with persistent lymphocyte activation represents the most convincing explanation for lymphoproliferation. Gastric MALT lymphoma is strictly associated with Helicobacter pylori infection and various eradicating protocols, developed due to increasing antibiotic resistance, represent the first line therapy for gastric MALT...
2016: Mediterranean Journal of Hematology and Infectious Diseases
Faisal Inayat, Jason K Law, Patricia M Myers-Gurevitch, Surya V Seshan, Alan S Perlman
Minimal change disease (MCD) in association with low-grade extra-nodal marginal zone B-cell lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) (MALT lymphoma) is a rare clinicopathologic entity. We report a 68-year-old male who presented with nephrotic range proteinuria as the first manifestation of underlying MZL, confirmed with standard set of investigations. Being a steroid non-responder, he was treated with rituximab demonstrating a marked response with resolution of proteinuria. However, he relapsed after 3 months...
March 2016: Clinical Nephrology
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