Clonal relationship of marginal zone lymphoma and diffuse large B-cell lymphoma in Sjogren's syndrome patients: case series study and review of the literature.

Published on Mar 1, 2020in Rheumatology International2.631
· DOI :10.1007/S00296-019-04470-X
Yulia V. Sidorova2
Estimated H-index: 2
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Abstract
The occurrence of diffuse large B-cell lymphoma (DLBCL) in the course of Sjogren’s syndrome (SS) is considered to be equally related either to the development of DLBCL de novo or to the transformation from marginal zone lymphoma (MZL). However, the question of possible clonal relationship between MZL and DLBCL in the group of SS patients remains open. Here we present the data concerning 194 patients with lymphoma complicated SS followed up at Nasonova Research Institute of Rheumatology during the last 22 years. Molecular analysis of tumor cells was performed for 6 SS patients who had developed both MZL and DLBCL. To assess clonal relationship between each of the tumor pairs immunoglobulin heavy chain (IGH) gene rearrangements were identified according BIOMED-2 protocol by means of multiplex polymerase chain reaction followed by GeneScan fragment analysis. Despite different localization MZL and DLBCL were clonally related in five tumor pairs. The median time to transformation was 11 months (range 0–78 months). MZL and DLBCL were clonally related in most cases from our cohort of SS patients. No statistically significant difference in survival between patients with DLBCL transformed from MZL and patients with de novo DLBCL was found in the cohort of SS patients investigated.
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#1Alessia Alunno (University of Perugia)H-Index: 37
#2Maria Comasia Leone (University of Perugia)H-Index: 8
Last. Francesco Carubbi (University of L'Aquila)H-Index: 31
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Primary Sjogren’s syndrome (pSS) is a systemic autoimmune disease mainly affecting exocrine glands and leading to impaired secretory function. The clinical picture is dominated by signs and symptoms of mucosal dryness and the course of the disease is mild and indolent in the majority of cases. However, a subgroup of patients can also experience extraglandular manifestations that worsen the disease prognosis. pSS patients are consistently found to have a higher risk of developing non-Hodgkin lymp...
Source
WHO CLASSIFICATION OF TUMOURS OF HAEMATOPOIETIC AND LYMPHOID TISSUES , WHO CLASSIFICATION OF TUMOURS OF HAEMATOPOIETIC AND LYMPHOID TISSUES , کتابخانه مرکزی دانشگاه علوم پزشکی تهران
#1Carla Casulo (UR: University of Rochester)H-Index: 22
#2Jonathan W. Friedberg (UR: University of Rochester)H-Index: 72
Abstract 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. Ho...
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#1Akashi FujitaH-Index: 2
#2Masahiro TajikaH-Index: 35
Last. Yasumasa NiwaH-Index: 51
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: API2-MALT1 translocation-positive gastric marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT) lymphoma is thought to transform to diffuse large B-cell lymphoma (DLBCL) rarely. A 69-year-old man presented with epigastralgia. Esophagogastroduodenoscopy showed multiple ulcerations in the stomach. Endoscopic biopsies revealed MALT lymphoma, with Helicobacter pylori infection. The patient underwent eradication therapy with no improvement, and was thereafter followed without addition...
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This study analysed incidence, patient outcome, immunophenotype and prognostic factors of histological transformation (HT) from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) to diffuse large B-cell lymphoma (DLBCL) in 467 patients (median age, 61 years). The primary sites of MALT lymphoma were the stomach (43%), ocular adnexa (25%), lung (8%), systemic (8%) and other tissues (16%). HT occurred in 8% of MALT lymphomas. Risk of HT by 15 years was 5%:...
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Patients with low-grade (clinically indolent) lymphomas are at risk to undergo transformation to high-grade (clinically aggressive) lymphoma, although transformation only occurs in a subset of patients. When transformation occurs it is a critical event that determines the course of disease and is associated with unfavorable patient outcomes. Accurate detection of transformation, predictive biomarkers, and identification of specific molecular pathways implicated in the pathobiology of transformat...
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#1Annarita Conconi (University of Eastern Piedmont)H-Index: 30
#2Silvia Franceschetti (University of Eastern Piedmont)H-Index: 24
Last. Emanuele ZuccaH-Index: 87
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Histologic transformation (HT) is a poorly understood event in patients with marginal zone lymphoma (MZL). The aim of this study was to analyze incidence and risk factors for HT in a large series of MZL patients.The studied cohort included 340 MZL patients diagnosed and treated between 1995 and 2012: 157 extranodal MZLs [mucosa-associated lymphoid tissue (MALT) lymphoma, 46%], 85 splenic MZLs (SMZLs, 25%) and 37 nodal MZLs (NMZLs, 11%). Sixty-one patients (18%) had bone marrow infiltration at pr...
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#1Gaetane Nocturne (French Institute of Health and Medical Research)H-Index: 21
#2Xavier Mariette (French Institute of Health and Medical Research)H-Index: 102
Abstract Primary Sjogren Syndrome (pSS) is an autoimmune disease associated with an increased risk of lymphoma. Lymphomas complicating pSS are mostly low-grade B cell non-Hodgkin lymphomas, predominantly of marginal zone histological type. Mucosal localization is predominant, notably mucosa-associated lymphoid tissue lymphomas. Lymphomas often develop in organs where pSS is active, such as salivary glands. Germinal centre (GC)-like structures, high TNFSF13B (BAFF) and Flt3-ligand (FLT3LG) levels...
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#1John G. Routsias (UoA: National and Kapodistrian University of Athens)H-Index: 26
#2John D. Goules (UoA: National and Kapodistrian University of Athens)H-Index: 3
Last. Michael Voulgarelis (UoA: National and Kapodistrian University of Athens)H-Index: 30
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Objectives: Sjogren’s syndrome (SS), a chronic autoimmune disorder, particularly compromises the function of exocrine glands. Its association with lymphoma is well documented. Our aim was to systematically review the molecular, clinical, histopathologic, and therapeutic aspects of these SS-related malignant lymphoproliferations. Methods: The literature was searched for original articles published between 1968 and 2012 focusing on the risk factors for lymphoma development in Sjogren’s syndrome us...
Source
#1Willemina R. R. Geurts-Giele (EUR: Erasmus University Rotterdam)H-Index: 11
#2Ingrid L. M. Wolvers-Tettero (EUR: Erasmus University Rotterdam)H-Index: 32
Last. Anton W. Langerak (EUR: Erasmus University Rotterdam)H-Index: 58
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Last. Westley H. Reeves (UF: University of Florida)H-Index: 61
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Sjogren's Syndrome (SS) is a chronic autoimmune disorder characterized by focal mononuclear cell infiltrates that surround the ducts of the exocrine glands, impairing the function of their secretory units. Compared to other autoimmune disorders, SS is associated with a notably high incidence of non-Hodgkin lymphoma (NHL) and more frequently mucosa associated lymphoid tissue (MALT) lymphoma, leading to increased morbidity and mortality rates. High risk features of lymphoma development include sys...
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IgG4-related disease (IgG4-RD) is a newly classified but poorly understood immune-medicated systemic disease. It causes potential fibroinflammation in one or more organs, characterized by tumescent organs and marked IgG4-positive plasma cells infiltration in the affected tissues. There have been a few cases revealing close relationship between IgG4-RD and formation of B cell lymphoma. Diffuse large B cell lymphoma (DLBCL) and extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid...
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