scholarly journals Clinical and Histological Spectrum of Primary Cutaneous B-Cell Lymphoma in an Ethnically-Diverse Group of Patients: A Case Series

2021 ◽  
Vol 5 (2) ◽  
pp. 160-165
Author(s):  
Sahira Farooq ◽  
Madeline Frizzell ◽  
Emily Limmer ◽  
Ritu Swali ◽  
Stephen Tyring
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
Case Series ◽  
Ethnically Diverse ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Cutaneous Lymphomas ◽  
Large B Cell ◽  
Cutaneous B Cell Lymphoma

Primary cutaneous lymphomas, which are diagnosed with only skin involvement, can be of B or T cell origin. Due to their varied origin as well as their diverse presentations, these groups have been divided further into more descriptive and representative groups. The subtypes are primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous diffuse large B cell lymphoma, leg type (PCDLBCL-LT) and intravascular large B-cell lymphoma. Here, we present the cases of three ethnically diverse patients with distinct clinical and histological forms of cutaneous B cell lymphoma.

scholarly journals A Unique Presentation of Cutaneous Diffuse Large B-Cell Lymphoma

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Mary Awad ◽  
Erik Holzwanger ◽  
Sandeep Jubbal
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
Survival Rates ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Subcutaneous Nodules ◽  
Large B Cell Lymphoma ◽  
Cutaneous Lymphomas ◽  
Large B Cell

Cutaneous B-cell lymphomas (CBCL) are rare heterogeneous neoplastic diseases composing about 22.5% of all cutaneous lymphomas. These diseases can be divided into primary and secondary cutaneous variants with primary cutaneous B-cell lymphoma (PCBCL) divided into three distinct entities including primary cutaneous marginal zone lymphoma, primary cutaneous follicle center lymphoma, and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT). Secondary cutaneous diffuse large B-cell lymphoma (CDLBCL) and PCDLBCL, LT are more aggressive neoplasms compared to the aforementioned CBCL with survival rates of 37% and 50% after 5 years, respectively. CDLBCL can present as cutaneous or subcutaneous nodules, papular lesions, or indurated plaques. Here, we present a case of CDLBCL of an 88-year-old female that was mistaken for lower extremity cellulitis with phlegmon. Our patient failed two courses of antibiotic therapy as an outpatient and received a third as an inpatient before a cutaneous biopsy clinched the diagnosis.

Transformation of a primary cutaneous marginal zone lymphoma into a cutaneous diffuse large B-cell lymphoma

Piel ◽  
2017 ◽  
Vol 32 (5) ◽  
pp. 314-316
Author(s):  
Carolina Areán ◽  
Alicia Córdoba ◽  
Juan García ◽  
Amaia Larumbe
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

bcl-2 protein expression in primary cutaneous large B-cell lymphoma is site-related.

1998 ◽  
Vol 16 (6) ◽  
pp. 2080-2085 ◽  
Author(s):  
F A Geelen ◽  
M H Vermeer ◽  
C J Meijer ◽  
S C Van der Putte ◽  
E Kerkhof ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
B Cell Lymphomas ◽  
European Organization ◽  
Clinical Behavior ◽  
Large B Cell Lymphoma ◽  
Cutaneous Lymphomas ◽  
Distinct Disease ◽  
Large B Cell

PURPOSE Primary cutaneous large B-cell lymphoma (PCLBCL) that presents on the leg has recently been recognized as a distinct disease entity. These lymphomas have a reduced disease-free survival and a worse prognosis as compared with the more common, morphologically similar PCLBCL that present on the head or trunk. Studies in noncutaneous diffuse large B-cell lymphomas suggest a relationship between the expression of bcl-2 protein and clinical behavior. In the present study, we investigated whether these two groups of PCLBCL differ in the expression of bcl-2 protein and the presence of t(4;18), known as one of the causes of bcl-2 overexpression. PATIENTS AND METHODS Paraffin sections from pretreatment biopsies of 14 PCLBCLs of the head or trunk and nine PCLBCLs of the legs were investigated for expression of bcl-2 protein using immunohistochemistry, and for the presence of the 14;18 translocation using polymerase chain reaction (PCR) amplification with primers against both the major breakpoint region (mbr) and the minor cluster region (mcr) of bcl-2. For reasons of comparison, nine secondary cutaneous large B-cell lymphomas (SCLBCLs) were also studied. RESULTS Expression of bcl-2 protein was found in all nine PCLBCLs of the leg and in all nine SCLBCLs, but not in any of the 14 PCLBCLs on the head and trunk. The t(14;18) was only detected in two of seven SCLBCLs, but not in the five PCLBCLs of the leg or the eight PCLBCLs on the head or trunk studied. CONCLUSION The striking differences in bcl-2 expression between PCLBCL of the head or trunk and PCLBCL on the leg suggest that bcl-2 expression is site-related and may contribute to the different clinical behavior between these two groups of lymphomas. In addition, they underscore that PCLBCL on the head and trunk and PCLBCL on the leg are distinct disease entities, as recently recognized in the European Organization for Research and Treatment of Cancer (EORTC) classification for primary cutaneous lymphomas.

Primary and Secondary Ocular Adnexal Lymphoma: A Single Institutional Experience

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4956-4956
Author(s):  
Darcie Deaver ◽  
Kenneth S. Zuckerman ◽  
Celeste M. Bello ◽  
Eduardo M. Sotomayor ◽  
Salvador Bruno ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Staging System ◽  
Low Grade ◽  
Time To Progression ◽  
Ocular Adnexal Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

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.

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

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1589-1589 ◽  
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.

Primary bone marrow diffuse large B cell lymphoma: a case series and review

2010 ◽  
Vol 90 (7) ◽  
pp. 791-796 ◽  
Author(s):  
Hung Chang ◽  
Yu-Shin Hung ◽  
Tung-Liang Lin ◽  
Po-Nan Wang ◽  
Ming-Chung Kuo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Primary Bone ◽  
Primary Bone Marrow ◽  
Large B Cell

scholarly journals Clinical manifestations of diffuse large B‑cell lymphoma in the skin and subcutaneous tissue a case series study

2020 ◽  
Vol 37 (5) ◽  
pp. 812-816
Author(s):  
Katarzyna Dulik ◽  
Grażyna Kamińska-Winciorek ◽  
Ryszard Swoboda ◽  
Anna Kwiatkowska-Pamula ◽  
Sebastian Giebel
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Subcutaneous Tissue ◽  
Clinical Manifestations ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Case Series Study ◽  
Series Study ◽  
Large B Cell

scholarly journals P024 The mighty mimic: intravascular large b-cell lymphoma masquerading as myositis: a case series

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Maria A Mirza
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Surrounding Tissue ◽  
Affected Area ◽  
Small Vessels ◽  
Large B Cell Lymphoma ◽  
Skin Biopsies ◽  
Large B Cell

Abstract Background/Aims  Intravascular large b-cell lymphoma (IVLBCL) is a rare and aggressive form of extranodal diffuse lymphoma which due to its heterogeneous manifestations is often diagnosed at post mortem. The disease is typically contained within the small vessels thus sparing the surrounding tissue. Indeed, the presentation of the disease will depend on the organ involved with classical involvement of the central nervous system and skin. Methods  We herein report two compelling cases of patients presenting with features of myositis, only to find on muscle biopsy evidence of IVBCL. Biopsy of the affected area and indeed random skin biopsies (RSB) may be a fundamental tool in establishing this elusive diagnosis. Results  Features of the two cases are discussed, along with their various presentations, investigations, treatment and overall trajectory. Conclusion  IVBCL is a rare disease that can present in various ways and often a delay in reaching a diagnosis leads to patient deterioration and death. A multidisciplinary approach involving the microbiologists, rheumatologists and haematologists led to a timely identification of the disease and subsequent management in these two cases. As the disease is characteristically contained within the blood vessels, the diagnosis is normally not confirmed from peripheral blood tests or even bone marrow biopsy. Biopsy of the affected area is what revealed the diagnosis in our two cases. Random skin biopsies may be very useful in establishing the diagnosis when suspecting IVBCL. Treatment may be very effective in controlling the disease as demonstrated by these two cases. Disclosure  M.A. Mirza: None.

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