Nodal marginal zone lymphoma

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Arachidonate 5-lipoxygenase (ALOX5) is a cardinal enzyme in the synthesis of leukotrienes, which are powerful immune-regulating lipid mediators. We previously reported that ALOX5 is preferentially expressed in B lymphocytes in the mantle zone of human lymphoid tissue. In the context of physiological relevance, the loss of the Alox5 gene in mice significantly impairs the development of follicular B helper T cells and antibody production. However, ALOX5 expression in B-cell lymphomas has not been investigated in detail...

Pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma are pediatric B cell lymphomas with similar clinical characteristics but distinct histological features. We investigated the differences between pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma by comparing their histological and molecular characteristics. A total of 5 pediatric-type follicular lymphoma and 11 pediatric nodal marginal zone lymphoma patients were included in the study. In the histological review, 5 of the 16 cases showed overlapping morphological features of pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma; hence, they were reclassified as "mixed type...

Marginal zone lymphomas (MZLs) represent about 7% of B-cell non-Hodgkin lymphomas and include 3 different subtypes-namely, extranodal (EMZL), nodal, and splenic (SMZL). The initial assessment requires specific diagnostic and staging procedures depending on organ-related peculiarities. In particular, although positron emission tomography/computed tomography was not initially recommended, recent data have reassessed its role in the routine staging of MZL, especially when only localized treatment is planned or there is a suspicion of histologic transformation...

Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are indolent mature B-cell neoplasms with variable clinical presentation and distinct histopathologic features. Recent advances in the biology and molecular characteristics of these lymphomas have further expanded our understanding of the heterogeneous nature of these lymphomas, with increasing recognition of specific disease entities within the broader categories of FL and MZL. Here, we discuss the conclusions of the 2022 International Consensus Classification of Mature Lymphoid Neoplasms (2022 ICC) dealing with FL, and review differences with the proposed WHO 5th Edition classification...

Nodal Marginal Zone Lymphoma(NMZL) is an indolent lymphoma with a very low clinical incidence and is sometimes difficult to differentiate diagnostically from Lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia (LPL/WM). NMZL with elevated monoclonal immunoglobulin M (IgM) is even rarer. Nontraumatic chylothorax can be seen in aggressive lymphoma, which often happens with chest tightness and dyspnea as the primary clinical manifestation. We reported the first case of monoclonal IgM elevated NMZL complicated by atypical nontraumatic chylothorax...

Background: Survival rates are usually used to evaluate the effect of cancer treatment and prevention. This study aims to analyze the 5-year relative survival of non-Hodgkin lymphoma (NHL) in United States using population-based cancer registry data. Methods: A period analysis was used to evaluate the improvement in long-term prognosis of patients with NHL from 2004 to 2018, and a generalized linear model was developed to predict the 5-year relative survival rates of patients during 2019-2023 based on data from the SEER database stratified by age, sex, race and subtype...

PURPOSE: Nodal marginal zone lymphoma (NMZL) localized to a single lymphatic region (i.e. Stage I) is a relatively rare diagnosis. Current guidelines permit these patients to be either observed or treated with systemic therapy (ST), radiotherapy (RT), or both modalities. The prognostic impact of ST or RT compared to observation has not been established. The purpose of this study is to assess the prognostic impact of therapy in Stage I NMZL. MATERIALS AND METHODS: The National Cancer Database (NCDB) was queried (2004-2018) for all patients with Stage I NMZL...

Diffuse large B-cell lymphoma (DLBCL) is the most common type of lymphoma worldwide, accounting for about 40% of cases. The role of cytokines in the pathogenesis of lymphomas has been rarely addressed, although cytokines have a close immunological relationship with lymphocytes. We observed overexpression of interleukin (IL)-20 in reactive germinal centres (GCs) leading us to hypothesise that IL-20 may play a role in lymphomagenesis. In this study, we surveyed for IL-20 expression in various types of lymphoma and found that IL-20 was expressed most frequently in follicular lymphoma (94%), but also in Burkitt lymphoma (81%), mantle cell lymphoma (57%), nodal marginal zone lymphoma (56%), Hodgkin lymphomas (50%), small lymphocytic lymphoma (50%) and diffuse large B-cell lymphoma (DLBCL, 48%)...

CONTEXT: Treatment options for patients with R/R FL or MZL are limited. Tafasitamab is an Fc-engineered humanized monoclonal antibody against CD19, which is broadly expressed in FL and MZL and regulates B-cell proliferation. Tafasitamab monotherapy showed clinical activity in a phase 2a study (NCT01685008), with an ORR of 29% (n/N=10/34) and 33% (n/N=3/9) in patients with FL and MZL, respectively. In an ongoing phase 2 study (L-MIND, NCT02399085), tafasitamab plus lenalidomide (LEN) followed by tafasitamab alone demonstrated an ORR of 57...

CONTEXT: CD83 is expressed by mature dendritic cells and activated lymphocytes but not by other peripheral blood cells. Some tumor cells have high expression of CD83 on the cell surface, such as Hodgkin lymphomas (HLs), diffuse large B-cell lymphoma, adult acute lymphoblastic leukemia (ALL), gastric extra-nodal marginal zone lymphomas, and lung cancer cells. OBJECTIVES: To determine the prognostic role of CD83 in adult ALL and correlated it with clinical outcome...

Extra-nodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) is uncommon and difficult to diagnose due to varied clinical presentations and endoscopic appearances masquerading as other pathology. Rarely, it has been associated with acute upper gastrointestinal (GI) bleeding. We report on a 60-year-old male who presented with an acute upper GI bleed and endoscopic findings suggestive of isolated gastric varices (GV), ultimately determined to be MALT lymphoma. Complete remission was achieved with radiation therapy, with no recurrence at a 12-month follow-up...

Introduction: Sarcoidosis is a chronic systematic inflammatory disorder of unknown etiology. Marginal zone lymphoma is a well-varied family of lymphomas that makes up to 10% of the overall non-Hodgkin's lymphomas. The relationship between sarcoidosis and multiple diseases was mentioned repeatedly in the literature, whereas the relationship between sarcoidosis and marginal zone lymphoma was found rare. This case presents a 56-years-old man who was diagnosed with sarcoidosis after treatment of marginal zone lymphoma...

Pediatric-type follicular lymphoma (PTFL) and pediatric nodal marginal zone lymphoma (PNMZL) are rare pediatric-type indolent B-cell lymphomas (PedIBCL) that differ clinicopathologically from their adult counterparts. Accurate diagnosis is important to avoid overtreatment but is often challenging. The mutational landscape of PTFL is known and may aid diagnosis, but the genetic features of PNMZL are not well understood. We analyzed 21 cases of PedIBCL according to their clinicopathologic findings and classified them into PTFL (n=11), PNMZL (n=2), and "mixed type" tumors (n=8) showing ambiguous histology...

Treatment of non-Hodgkin lymphoma (NHL) in general has improved over the years with the emergence of the monoclonal antibodies (MAB) therapy. NHL is divided into B cell NHL and T cell NHL. Treatment of NHL was based on the subtype of NHL and its staging. NHL is divided into aggressive and indolent NHL (iNHL). Subtypes of iNHL include: Follicular lymphoma (FL), Marginal zone lymphoma (MZL), Chronic lymphocytic leukemia/small-cell lymphocytic lymphoma (CLL/SLL), Gastric mucosa-associated lymphoid tissue (MALT) lymphoma, Lymphoplasmacytic lymphoma, Waldenström macroglobulinemia, Nodal marginal zone lymphoma (NMZL), Splenic marginal zone lymphoma (SMZL)...

Pediatric nodal marginal zone lymphoma (PNMZL) is an uncommon B-cell neoplasm affecting mainly male children and young adults. This indolent lymphoma has distinct characteristics that differ from those of conventional nodal marginal zone lymphoma (NMZL). Clinically, it exhibits overlapping features with pediatric-type follicular lymphoma (PTFL). To explore the differences between PNMZL and adult NMZL and its relationship to PTFL, a series of 45 PNMZL cases were characterized morphologically and genetically by using an integrated approach; this approach included whole-exome sequencing in a subset of cases, targeted next-generation sequencing, and copy number and DNA methylation arrays...

Histological transformation (HT) is an exceptionally uncommon and poorly understood event where a low-grade or indolent B-cell lymphoma transforms into a more aggressive entity, typically diffuse large B-cell lymphoma (DLBCL). The pathogenesis is unclear; however, HT is associated with a worse prognosis. This article reports a unique case of marginal zone lymphoma (MZL) limited to skin/subcutis (confirmed with PET-CT) that subsequently developed DLBCL, followed by nodal MZL. We explored phenotypic, molecular genetic, and cytogenetic findings in subcutaneous MZL with HT to DLBCL and subsequent progression to systemic MZL...

RATIONALE: Nodal marginal zone B-cell lymphoma (NMZL) is a relatively rare indolent lymphoma that is the least common subtype of marginal zone B-cell lymphoma. Furthermore, coexistence of lymphoma and multiple myeloma (MM) is rare. Here, we report a case of the coexistence of NMZL and MM. PATIENT CONCERNS: The patient was a 66-year-old man with pain in the left rib and lower left back. Enlarged lymph nodes were palpable in the cervical and inguinal regions, the biggest of which was 4...

Marginal zone lymphoma (MZL) is a heterogenous group of indolent non-Hodgkin lymphomas (NHLs). Three subtypes are recognized based on the site of involvement: extranodal MZL, splenic MZL, and nodal MZL. MZL represents 7% of all mature NHLs that exhibit geographical variability in their incidence and association with infectious agents. Each MZL subtype is characterized by unique biology, clinical presentation, therapeutic approach, and natural history. Recent findings have improved risk stratification of patients at diagnosis and after frontline therapy; however, these data are not incorporated into treatment decisions or selections of therapeutic agents...

Lymphomas of the breast are rare neoplasms that arise from breast lymphoid tissue and are characterised by neoplastic B or T cells. Breast lymphomas arising from B cells include, but are not limited to, diffuse large B cell lymphoma, follicular lymphoma, extra-nodal marginal zone lymphoma and Burkitt lymphoma. Anaplastic large cell lymphoma (ALCL) is of a T cell origin and both anaplastic lymphoma kinase (ALK)-positive and ALK-negative presentations have been noted in the breast. In addition, there is a more recently identified presentation of ALK-negative ALCL that arises around textured breast implants and is usually confined to a periprosthetic fibrous capsule...