scholarly journals Lymphomas in Patients With Sjögren's Syndrome Are Marginal Zone B-Cell Neoplasms, Arise in Diverse Extranodal and Nodal Sites, and Are Not Associated With Viruses

Blood ◽  
1997 ◽  
Vol 90 (2) ◽  
pp. 766-775 ◽  
Author(s):  
Bruno Royer ◽  
Dominique Cazals-Hatem ◽  
Jean Sibilia ◽  
Felix Agbalika ◽  
Jean-Michel Cayuela ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
P53 Protein ◽  
B Cell Lymphoma ◽  
Lymphoid Tissues ◽  
Low Grade ◽  
Human Herpes Virus 8 ◽  
Barr Virus ◽  
P53 Antibodies ◽  
Histological Transformation

Abstract The occurrence of non-Hodgkin's lymphoma (NHL) is the most serious complication of Sjögren's syndrome (SS). We performed a study of 16 NHLs occurring in patients with an underlying SS. These lymphomas arose not only in salivary glands (7 cases) but also in other mucosal extranodal sites (the stomach [4 cases], the lung [3 cases], the skin [3 cases], the buccal mucosa [1 case], the thymus [1 case]) and in nodal sites (8 cases). Low-grade marginal zone lymphomas (MZL) were diagnosed in 12 of the 16 patients, 9 of mucosa-associated lymphoid tissues (MALT) type in mucosal sites and 3 exclusively nodal. The 4 other patients presented with a high-grade B-cell lymphoma that was probably a histological transformation of an underlying low-grade MZL at least in 3 of the cases involving skin, stomach, and parotid, respectively. A t(14; 18) translocation was detected in 1 of 8 lymphomas tested. We detected serum anti-p53 antibodies in 2 of the 14 studied patients. p53 protein was detected in 1 of 11 lymphomas tested. LMP protein and Eber RNAs of Epstein-Barr virus (EBV) were not detected in the 16 NHL biopsies. Using polymerase chain reaction, EBV was never detected except in 1 of 4 parotid lymphomas. No human T-lymphotropic virus 1 or human herpes virus 8 DNAs were detected in NHL biopsies. None of the patients had hepatitis C virus infection found using serological methods. Chemotherapy was usually efficient. In conclusion, lymphomas occurring in patients with an underlying SS are in most cases MZL. These lymphomas are not associated with viruses known to be present in other types of lymphomas. Some of the translocations or mutations of oncogenes or antioncogenes described in other lymphomas are detected in SS-associated lymphomas.

scholarly journals Lymphomas in Patients With Sjögren's Syndrome Are Marginal Zone B-Cell Neoplasms, Arise in Diverse Extranodal and Nodal Sites, and Are Not Associated With Viruses

Blood ◽  
1997 ◽  
Vol 90 (2) ◽  
pp. 766-775 ◽  
Author(s):  
Bruno Royer ◽  
Dominique Cazals-Hatem ◽  
Jean Sibilia ◽  
Felix Agbalika ◽  
Jean-Michel Cayuela ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
P53 Protein ◽  
B Cell Lymphoma ◽  
Lymphoid Tissues ◽  
Low Grade ◽  
Human Herpes Virus 8 ◽  
Barr Virus ◽  
P53 Antibodies ◽  
Histological Transformation

The occurrence of non-Hodgkin's lymphoma (NHL) is the most serious complication of Sjögren's syndrome (SS). We performed a study of 16 NHLs occurring in patients with an underlying SS. These lymphomas arose not only in salivary glands (7 cases) but also in other mucosal extranodal sites (the stomach [4 cases], the lung [3 cases], the skin [3 cases], the buccal mucosa [1 case], the thymus [1 case]) and in nodal sites (8 cases). Low-grade marginal zone lymphomas (MZL) were diagnosed in 12 of the 16 patients, 9 of mucosa-associated lymphoid tissues (MALT) type in mucosal sites and 3 exclusively nodal. The 4 other patients presented with a high-grade B-cell lymphoma that was probably a histological transformation of an underlying low-grade MZL at least in 3 of the cases involving skin, stomach, and parotid, respectively. A t(14; 18) translocation was detected in 1 of 8 lymphomas tested. We detected serum anti-p53 antibodies in 2 of the 14 studied patients. p53 protein was detected in 1 of 11 lymphomas tested. LMP protein and Eber RNAs of Epstein-Barr virus (EBV) were not detected in the 16 NHL biopsies. Using polymerase chain reaction, EBV was never detected except in 1 of 4 parotid lymphomas. No human T-lymphotropic virus 1 or human herpes virus 8 DNAs were detected in NHL biopsies. None of the patients had hepatitis C virus infection found using serological methods. Chemotherapy was usually efficient. In conclusion, lymphomas occurring in patients with an underlying SS are in most cases MZL. These lymphomas are not associated with viruses known to be present in other types of lymphomas. Some of the translocations or mutations of oncogenes or antioncogenes described in other lymphomas are detected in SS-associated lymphomas.

Molecular analysis of low grade extranodal marginal zone B-cell lymphoma by alumorph genotyping

2000 ◽  
Vol 118 (4) ◽  
pp. A1385
Author(s):  
Michele De Boni ◽  
Francesco Bertoni ◽  
Roman Mullenbach ◽  
Enrico Roggero ◽  
Angelo Bellumat ◽  
...  
Keyword(s):  
B Cell ◽  
Molecular Analysis ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Low Grade

scholarly journals Extranodal marginal zone non Hodgkin's lymphoma of the lung: A ten-year experience

2011 ◽  
Vol 68 (2) ◽  
pp. 150-154 ◽  
Author(s):  
Violeta Milosevic ◽  
Andrija Bogdanovic ◽  
Snezana Jankovic ◽  
Maja Perunicic-Jovanovic ◽  
Biljana Mihaljevic
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Eastern Cooperative Oncology Group ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
Chop Regimen ◽  
Female Ratio ◽  
Balt Lymphoma

Background/Aim. Bronchus-associated lymphoid tissue (BALT) lymphoma is a rare subtype of low grade marginal zone B cell lymphoma representing 10% of all MALT lymphomas. The purpose of this study was to analyze the outcome of this group of patients comparing prognostic parameters and therapy modalities. Methods. A total of eight patients with BALT lymphoma had diagnosed between January 1998 - April 2008 at the Institute of Hematology, Clinical Center of Serbia, Belgrade, and they were included in this retrospective analysis. Results. Male/female ratio was 2/6, the median age was 64 years (range 37-67 years). Six patients had nonspecific respiratory symptoms and all of them had B symptoms. The patients were seronegative for HIV, HCV and HBsAg. Three patients had Sjogren's syndrome, rheumatoid arthritis and pulmonary tuberculosis, respectively. Seven patients were diagnosed by transbronchial biopsy and an open lung biopsy was done in one patient. Patohistological findings revealed lymphoma of marginal zone B cell lymphoma: CD20+/CD10-/CD5-/CyclinD1- /CD23-/IgM- with Ki-67+<20% of all cells. According to the Ferraro staging system, five patients had localized disease (CS I-IIE) and three had stage IVE; bulky tumor mass had 3 patients. All patients had Eastern Cooperative Oncology Group (ECOG) performance status (PS) 0 or 1. Five patients received monochemotherapy with chlorambucil and 3 were treated with CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone). A complete response (CR) was achieved in 5 patients and a partial response (PR) in 3 of them, treated with chlorambucil monotherapy and CHOP regimen. All patients were alive during a median follow-up period of 49 months (range 6- 110 months). Three patients relapsed after monochemotherapy into the other extranodal localization. They were treated with CHOP regimen and remained in stable PR. Conclusion. BALT lymphoma tends to be localised disease at the time of diagnosis, responds well to monochemotherapy with chlorambucil and has a favourable prognosis.

Diagnosis and management of cold agglutinin disease associated with low-grade B-cell lymphoma in a patient receiving pembrolizumab for lung cancer

2021 ◽  
Vol 14 (8) ◽  
pp. e243751
Author(s):  
Nabin Raj Karki ◽  
Peyton McElhone ◽  
Natasha Savage ◽  
Nagla Abdel Karim
Keyword(s):  
Lung Cancer ◽  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Titre ◽  
Cold Agglutinin ◽  
Low Grade ◽  
Cold Agglutinin Disease ◽  
Cold Room

A 65-year-old with non-small cell lung cancer developed autoimmune haemolytic anaemia while receiving pembrolizumab containing chemoimmunotherapy. Initially thought to be due to pembrolizumab induced haemolysis, he was treated with steroids, and pembrolizumab was held. Haemolysis was refractory to steroids and blood was observed to agglutinate in cold room temperatures. Cold agglutinins in high titre and monoclonal serum IgM kappa protein were detected. Bone marrow biopsy showed marginal zone lymphoma confirming low grade B-cell lymphoma causing cold agglutinin disease. B-cell depletion by rituximab stopped haemolysis, and pembrolizumab was safely continued for lung cancer.

Clinicopathologic and Genetic Profile of Intracranial Marginal Zone Lymphoma: A Primary Low-Grade CNS Lymphoma That Mimics Meningioma

2005 ◽  
Vol 23 (24) ◽  
pp. 5718-5727 ◽  
Author(s):  
Pang-hsien Tu ◽  
Caterina Giannini ◽  
Alexander R. Judkins ◽  
Jason M. Schwalb ◽  
Richard Burack ◽  
...  
Keyword(s):  
Marginal Zone ◽  
Primary Cns Lymphoma ◽  
B Cell Lymphoma ◽  
Cns Lymphoma ◽  
Genetic Profile ◽  
Low Grade ◽  
Barr Virus ◽  
Genetic Features ◽  
Trisomy 3 ◽  
Epstein Barr

Purpose Although rare overall, marginal zone B-cell lymphoma (MZBCL) is the most common primary low-grade CNS lymphoma reported in the literature. The aim of this study is to elucidate the biology and genetic features of this unusual tumor. Patients and Methods Fifteen CNS MZBCLs were studied clinically, pathologically, and genetically, including fluorescent in situ hybridization analyses with commercially available MALT1 and IgH break-apart and centromere 3, 7, 12, and 18 probes. Results CNS MZBCLs preferentially affect middle-aged women (female-to-male ratio, 4:1), with 93% presenting as dural-based masses mimicking meningioma. Ten patients with 1 to 7.6 years of follow-up after diagnosis showed no evidence of disease after radiation and/or chemotherapy. Like MZBCLs outside of the CNS, they consisted of CD20+, CD3− small B lymphocytes with varying degrees of plasmacytic differentiation and predominantly κ light-chain restriction (78%). Lymphoid follicles with follicular colonization were seen in three patients and deposition of amyloid was noted in samples from two patients, one of which was tumefactive. Neither Bcl-6 protein nor Epstein-Barr virus–encoded RNA was expressed. Trisomy 3 was detected in six of 12 patients, with no rearrangements of MALT1 or IgH and no trisomies of 7, 12, or 18 detected. Conclusion Our data suggest that intracranial MZBCL is an indolent primary CNS lymphoma that typically presents as a meningioma-like dural-based mass. Trisomy 3, but not MALT1 or IgH translocation, is a common genetic abnormality that may contribute to the pathogenesis of this CNS lymphoma.

Gastric marginal zone B-cell lymphomas of MALT type develop along 2 distinct pathogenetic pathways

Blood ◽  
2002 ◽  
Vol 99 (1) ◽  
pp. 3-9 ◽  
Author(s):  
Petr Starostik ◽  
Jochen Patzner ◽  
Axel Greiner ◽  
Stephan Schwarz ◽  
Jörg Kalla ◽  
...  
Keyword(s):  
B Cell ◽  
Lymphoid Tissue ◽  
Gene Locus ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
High Grade ◽  
Clonal Proliferation ◽  
Large B Cell Lymphoma

Low-grade marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type can transform into high-grade diffuse large B-cell lymphoma (DLBCL). Up to 60% of the MALT lymphomas contain the recently described t(11;18). However, this translocation has not been detected in any DLBCL so far. To elucidate the pathogenesis of these tumors, microsatellite screening of 24 gastric MALT lymphomas was performed and the results were compared with aberrations detected in a previous study on gastric DLBCL. The most frequent aberration, found in 21% of the MALT lymphomas that were exclusively t(11;18)-negative cases, was amplification of the 3q26.2-27 region (harboring the locus of the BCL6 gene). Allelic imbalances in regions 3q26.2-27, 6q23.3-25, 7q31, 11q23-24, and 18q21 were shared by both MALT lymphoma and DLBCL. Loss of heterozygosity in regions 5q21 (APC gene locus), 9p21 (INK4A/ARF), 13q14 (RB), and 17p13(p53) and allelic imbalances in 2p16, 6p23, and 12p12-13 occurred exclusively in DLBCL. Only one of 10 t(11;18)-positive MALT lymphomas showed an additional clonal abnormality. These tumors thus display features of a clonal proliferation characterized by the presence of the t(11;18). However, they only rarely display secondary aberrations and do not seem to transform into DLBCL. In contrast, t(11;18)-negative MALT lymphomas show numerous allelic imbalances—some of them identical with aberrations seen in DLBCL—suggesting that this group is the source of tumors eventually transforming into high-grade DLBCL.

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