scholarly journals Transformation of low-grade follicular lymphoma with partial marginal zone differentiation: Two cases

2021 ◽  
Vol 13 (3) ◽  
Author(s):  
Karen Nalbandyan ◽  
Daniel Benharroch ◽  
Anna Gurevitch ◽  
Itai Levi

Two cases of low-grade follicular lymphoma, with marginal zone differentiation and/or with high proliferation rate in one of them, are reported with transformation into high grade B-cell and B-lymphoblastic lymphomas. The contribution of these features to the transforming process, although previously described, is infrequent, and has not been deciphered to date.

Blood ◽  
2002 ◽  
Vol 99 (1) ◽  
pp. 3-9 ◽  
Author(s):  
Petr Starostik ◽  
Jochen Patzner ◽  
Axel Greiner ◽  
Stephan Schwarz ◽  
Jörg Kalla ◽  
...  

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.


Diagnostics ◽  
2020 ◽  
Vol 10 (3) ◽  
pp. 157
Author(s):  
Anna Shestakova ◽  
Sherif Rezk ◽  
Dara Ghasemizadeh ◽  
Ali Nael ◽  
Xiaohui Zhao

Follicular lymphoma, the second most common non-Hodgkin lymphoma (NHL), primarily affects adults and shows an indolent clinical course. Rare cases of follicular lymphoma transform to a high-grade B-cell lymphoma with MYC and BCL2 rearrangements or “double-hit lymphoma”. Transformation to a “double-hit lymphoma” portends a worse prognosis and requires aggressive treatment. We report a comprehensive clinical, pathologic and radiographic review of a patient with previously undiagnosed low-grade follicular lymphoma that transformed into a “double-hit lymphoma”. The patient presented with a large heterogeneous mass 16 x 19 cm involving pancreatic head and neck and a mildly enlarged inguinal lymph node. Positron emission tomography (PET) study demonstrated Fluorodeoxyglucose (18F) (FDG)-avid peripancreatic mass. Tissue biopsy demonstrated a high-grade B-cell lymphoma with rearrangements t(14;18) and MYC, leading to the diagnosis of high-grade B-cell lymphoma with MYC and BCL2 rearrangements. Excisional biopsy of an inguinal lymph node demonstrated low-grade follicular lymphoma. Clonality studies demonstrated the same immunoglobulin clone V7-4 in inguinal lymph node and peripancreatic mass. Therefore, diagnosis of a high-grade B-cell lymphoma with MYC and BCL2 rearrangements that transformed from a low-grade follicular lymphoma was rendered. It is ultimately important to establish a tissue-based diagnosis at the different sites that are involved with lymphoma. Patient proceeded with the aggressive treatment with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab (EPOCH-R) treatment.


2021 ◽  
Vol 8 (11) ◽  
pp. 584-587
Author(s):  
Shima Kannadas ◽  
Rajeevan Kunnaruvath

BACKGROUND Extranodal marginal zone lymphomas are rare groups of B cell lymphomas that arise in mucosa associated lymphoid tissues of various sites. Strong association with autoimmunity and certain infections are its distinct features. Diagnosis is by histology with immunophenotyping alongside ruling out other low-grade lymphomas. In this study we wanted to analyse MALTomas with regard to their clinical features, relation to autoimmunity, histological and immunohistochemical profile. METHODS This is a descriptive study conducted from 2013 to 2018 at a tertiary care centre in North Kerala. Study sample included all cases of extranodal lymphomas obtained retrospectively from 2013 to 2017 and prospectively from 2017 to 2018. Blocks of all specimens were cut and stained with haematoxylin and eosin (H & E) and immunohistochemical markers. RESULTS Out of the 15 cases we analysed, the most common sites of involvement were gastrointestinal tract (40 %) followed by thyroid (27 %). Thyroid, which is a rarer site for MALT lymphoma, is detected as one of the frequent sites in this study. In our study, stomach was one of the less common site affected which is in contrast to other studies. Associated autoimmune disorder was detected in 20 % of cases. 46 % of cases show features of transformation to high grade lymphoma. CONCLUSIONS This study analysed the features of MALT lymphomas in different sites and found a strong association with autoimmune disorders. Rarer sites like thyroid were found to be more frequently involved and common sites like stomach was detected in only small percentage of cases. Although most cases are indolent low-grade lymphomas, possibility of transformation to a high-grade B cell lymphoma should always be considered when dealing with these cases. Utility of immunohistochemistry in ruling out other small cell lymphomas is also well demonstrated in this study. KEYWORDS Extranodal Marginal Zone Lymphoma, MALToma, Autoimmune Disorders, Immunohistochemistry


2000 ◽  
Vol 118 (4) ◽  
pp. A1385
Author(s):  
Michele De Boni ◽  
Francesco Bertoni ◽  
Roman Mullenbach ◽  
Enrico Roggero ◽  
Angelo Bellumat ◽  
...  

2011 ◽  
Vol 68 (2) ◽  
pp. 150-154 ◽  
Author(s):  
Violeta Milosevic ◽  
Andrija Bogdanovic ◽  
Snezana Jankovic ◽  
Maja Perunicic-Jovanovic ◽  
Biljana Mihaljevic

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.


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