Bilateral MALT-type ocular adnexal lymphoma with marginal zone lymphoma leukaemic cells and ophthalmological diffuse large B cell lymphoma

Abstract Abstract 4956 Background: The incidence of ocular adnexal lymphoma (OAL) is rare and usually presents in the setting of central nervous system (CNS) involvement. There are no rigid guidelines for the treatment of OAL, most probably because of the variety of characteristics of the disease. Objectives: To analyze clinical pathological features, therapy and outcomes of patients with primary and secondary OAL. Research Design and Methods: Retrospective chart review of 17 consecutive patients diagnosed with OAL at Moffitt Cancer Center from 2004–2011. Characteristics of the participants were median age 68 years, 15 (88%) white, 2 (12%) Hispanic, and 11 (65%) male. Chlamydia serology testing was negative in all patients tested. Secondary OAL patients were staged per the Ann Arbor Staging System, 2 (22%) stage III and 7 (78%) stage IV. The primary OAL patients were staged utilizing the TNM staging system for OALs (Coupland et al, Arch Pathol Lab Med, 2009). Six (75%) patients were stage T1 and 2 (25%) patients were stage T2. Results: Seventeen patients with a diagnosis of OAL were evaluated in our institution. Patients with OAL are commonly stratified into 2 groups, primary and secondary. Eight (50%) of the patients were diagnosed with primary OAL; of these there were 4 (50%) marginal zone, 3 (37%) diffuse large B cell, and 1 (12%) follicular lymphoma. Nine (50%) patients were diagnosed with secondary OAL; 4 (44%) marginal zone, 1 (11%) diffuse large B cell, 1 (11%) mantle cell, 1 (11%) CLL, and 2 (22%) progressed from low grade to diffuse large B cell lymphoma. In the primary OAL, radiation in combination with systemic chemotherapy was the preferred treatment in diffuse large B cell lymphoma and radiation was preferred in patients with low-grade lymphoma. In secondary OAL, systemic chemotherapy was the preferred treatment for aggressive lymphoma. The choice of systemic Rituximab, radiation, or observation was the preferred treatment of low-grade lymphoma. Aggressive primary OAL had a relapse rate of 2 (66%) patients with a median time to progression of 8 months. Aggressive secondary OAL demonstrated a relapse rate of 50% with median time to progression 6 months. All patients who experienced relapsed disease received salvage chemotherapy. No cases of relapse were observed in the low-grade, primary or secondary, OAL patients. Median duration of response in low-grade primary lymphoma was 6 months and the low-grade secondary lymphoma was 54 months. Conclusion: In our patient population diffuse large B cell lymphoma and marginal zone lymphoma were the most common diagnoses. Ocular adnexal lymphoma has been associated with the presence of CNS disease and it is estimated that 80–90% of patients diagnosed with OAL will experience progression to the CNS. Treatment depends on the extent of disease and the subtype of lymphoma that is histologically identified. Treatment may consist of involved field radiation in localized disease and has approximately less than 10% local recurrence rate. Intravitreal methotrexate and itraorbital injections of Rituximab or a combination of localized radiation and systemic high dose methotrexate are also options for treatment. In the event that there is systemic disease, single agent IV Rituximab or standard chemotherapy regimens such as CHOP-R or CVP-R in conjunction with ocular directed therapy. When disease is localized to the ocular compartment, the burden of disease is low and there is a greater chance of eradicating the disease. The delay in diagnosis increase the risk of CNS involvement and decreases overall survival. Disclosures: No relevant conflicts of interest to declare.

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Preferential Acquision of N-Glycosylation Sites in the VDJ Region in Germinal Center B-Cell-Like Difusse Large B-Cell Lymphoma

Blood ◽

10.1182/blood.v120.21.1589.1589 ◽

2012 ◽

Vol 120 (21) ◽

pp. 1589-1589 ◽

Cited By ~ 1

Author(s):

Miguel Alcoceba ◽

Elena Sebastián ◽

Ana Balanzategui ◽

Luis Marín ◽

Santiago Montes-Moreno ◽

...

Keyword(s):

B Cells ◽

B Cell ◽

Germinal Center ◽

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Marginal Zone Lymphoma ◽

Large B Cell Lymphoma ◽

Glycosylation Sites ◽

Large B Cell

Abstract Abstract 1589 Introduction: Acquired potentially N-glycosylation sites are produced by somatic hypermutation (SHM) in the immunoglobulin (Ig) variable region. This phenomenon is produced in ∼9% of normal B-cells and seems to be related to certain B-cell lymphoproliferative disorders (B-LPDs) such as follicular lymphoma (FL, 79%), endemic Burkitt lymphoma (BL, 82%) and diffuse large B-cell lymphoma (DLBCL, 41%). These data suggest that new potential N-glycosylation sites could be related to germinal center B (GCB)-LPDs. By contrast, in other B-LPDs, such as chronic lymphocytic leukemia (CLL), mantle cell lymphoma (MCL), MALT lymphoma, Waldenström macroglobulinemia (WM) or multiple myeloma (MM), these modifications have not been analyzed in deep. Aims: To evaluate the acquisition of potential N-glycosylation sites in B-LPDs, including immunohystochemical DLBCL subtypes (GCB and non-GCB) and specific non-GCB-LPDs, such as hairy cell leukemia (HCL), splenic marginal-zone lymphoma (SMZL), CLL, MCL, ocular extranodal marginal zone lymphoma (OAEMZL), MM and WM. Patients: A total of 953 sequences (203 from our group and 750 previously published sequences) of B-LPDs were included. Diagnosis distribution was as follows: DLBCL (n=235), MCL (n=235), CLL (n=166), MM (n=96), OAEMZL (n=82), SMZL (n=68), WM (n=38) and HCL (n=33). Methods: Acquired N-glycosylation sites were counted according to the sequence Asn-X-Ser/Thr, where X could be any amino acid except Pro. Natural motifs in germline sequences of IGHV1–08, IGHV4–34 e IGHV-5a were not considered. Fisher test was used to perform comparisons between groups. To distinguish DLBCL biological subtypes (GCB and non-GCB DLBCL), Hans' algorithm was used. Results: A total of 83 out of the 235 DLBCL cases acquired at least a new N-glycosylation site, a higher value than in normal B-cells (35% vs. 9%, p<0.0001). Higher incidence of these motifs in the group of GCB as compared to non-GCB DLBCL were observed (52% vs. 20%, p<0.0001). Those cases diagnosed of HCL, CLL, MCL, MM, WM, OAEMZL and SMZL presented a reduced number of new N-glycosylation sites, showing similar values than normal B-cells (range 3–18%, p=ns). Conclusions: We described for the first time the pattern of N-glycosylation in HCL, SMZL, OAEMZL and in the immunohystochemical DLBCL subtypes, where the GCB-DLBCL showed a higher number of new N-glycosylation sites with respect to non-GCB DLBCL and other non-GCB-LPDs. The presence of novel N-glycosylation sites in FL, BL and in GCB-DLBCL strongly suggests that these motifs are characteristic of the germinal center B-LPDs. Disclosures: No relevant conflicts of interest to declare.

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Diffuse large B cell lymphoma evolving from extranodal marginal zone lymphoma as the first presentation of complicated coeliac disease: case report and review of the literature

Journal of Hematopathology ◽

10.1007/s12308-011-0126-4 ◽

2011 ◽

Vol 6 (1) ◽

pp. 33-37

Author(s):

Mireille J. Hardie ◽

Isaac A. Thyer ◽

Tindaro Giardina ◽

Michael A. Platten ◽

Martin J. Hudson ◽

...

Keyword(s):

Case Report ◽

B Cell ◽

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Marginal Zone Lymphoma ◽

Review Of The Literature ◽

Large B Cell Lymphoma ◽

Disease Case ◽

Large B Cell

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A composite lymphoma combining a Hodgkin lymphoma and a marginal zone lymphoma transformed into a diffuse large B‐cell lymphoma

Clinical Case Reports ◽

10.1002/ccr3.1841 ◽

2018 ◽

Vol 6 (12) ◽

pp. 2341-2346 ◽

Cited By ~ 1

Author(s):

Claire Auditeau ◽

Olivier Lambotte ◽

Joffrey Feriel ◽

Thierry Lazure ◽

Ali Turhan ◽

...

Keyword(s):

B Cell ◽

Hodgkin Lymphoma ◽

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Marginal Zone Lymphoma ◽

Composite Lymphoma ◽

Large B Cell Lymphoma ◽

Large B Cell

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Gastrointestinal Lymphomas: Entities and Mimics

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2012-0211-cr ◽

2012 ◽

Vol 136 (8) ◽

pp. 865-870 ◽

Cited By ~ 6

Author(s):

Lauren B. Smith ◽

Scott R. Owens

Keyword(s):

B Cell ◽

Lymphoid Tissue ◽

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Marginal Zone Lymphoma ◽

Large B Cell Lymphoma ◽

Gastrointestinal Lymphomas ◽

Lymphoma Involvement ◽

Large B Cell

The gastrointestinal tract is the most common extranodal site of lymphoma involvement. Although B-cell lymphomas are by far the most frequent type found in this location, gastrointestinal lymphomas are a diverse group of neoplasms, many of which are characterized by distinctive clinicopathologic settings. Diffuse large B-cell lymphoma and marginal-zone lymphoma of mucosa-associated lymphoid tissue are commonly encountered, but other less-common entities can pose diagnostic challenges, mimicking both benign, reactive conditions and each other. We describe several different lymphoma subtypes, with a focus on frequently encountered challenges in differential diagnosis.

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CD5-Positive Marginal Zone Lymphoma: Clinicopathological Features And Survival Outcomes

10.21203/rs.3.rs-948719/v1 ◽

2021 ◽

Author(s):

Yaqin Xia ◽

Jurui Ge ◽

Zhenchang Sun ◽

Feifei Nan ◽

Wenjuan Wan ◽

...

Keyword(s):

B Cell ◽

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Marginal Zone Lymphoma ◽

Clinicopathological Features ◽

Survival Outcomes ◽

Large B Cell Lymphoma ◽

Significant Difference ◽

Large B Cell

Abstract Background: CD5 expression in diffuse large B-cell lymphoma has a poor prognosis but the prognostic value of CD5 expression in marginal zone lymphoma is undefined. Methods: Clinicopathological features, survival outcomes, and prognostic implications of marginal zone lymphoma were retrospectively analyzed in 204 patients. We classified patients into (i) CD5-positive marginal zone lymphoma (ii) CD5-negative marginal zone Lymphoma, Fisher's exact test was used to compare the CD5-positive and CD5-negative marginal zone lymphoma. Progression-free survival (PFS) and overall survival (OS) curves were summarized by Kaplan-Meier method and compared using the log-rank test, The Cox proportional hazard regression model was used for univariate and multivariate analyses. Results: CD5 expression is rare in marginal zone lymphoma, of 204 patients, only 48 (23.53%) had CD5-positive. Due to the characterized of slow growth and locally aggressive nature, the prognosis is favorable after treatment. at the end of the followup 179 patients were still alive,163 patients never progressed. The 5-year PFS and OS rates for marginal zone lymphoma were 65.10% and 77.30% respectively, the 5-year PFS and OS rates for CD5-positive marginal zone lymphoma were 64.80% and 84.10%, there is no significant difference between CD5-positive and CD5-negative ( P =0.829, P =0.521). Diffuse large B-cell lymphoma (DLBCL) transformation was pathologically indicated in 6 patients, of which 5(83.33%) patients were CD5-positive marginal zone lymphoma. Conclusion: CD5 expression in marginal zone lymphoma is not independently prognostic for PFS and OS. But CD5-positive marginal zone lymphoma seems more likely to transformation to diffuse large B-cell lymphoma.

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