How I Diagnose Primary Cutaneous Marginal Zone Lymphoma

2020 ◽  
Vol 154 (4) ◽  
pp. 428-449
Author(s):  
Sarah E Gibson ◽  
Steven H Swerdlow

Abstract Objectives Primary cutaneous marginal zone lymphoma (PCMZL) is 1 of the 3 major subtypes of primary cutaneous B-cell lymphoma. The diagnosis of PCMZL may be challenging, as the differential diagnosis includes benign cutaneous lymphoproliferations as well as other primary or secondary cutaneous B-cell or T-cell lymphomas. This review describes our approach to the diagnosis of PCMZL. Methods Two cases are presented that illustrate how we diagnose each of the 2 subtypes of PCMZL. The clinicopathologic features of PCMZL and the ways in which these cases can be distinguished from both benign and other neoplastic entities are emphasized. Results A definitive diagnosis of PCMZL requires the incorporation of histologic and immunophenotypic features, molecular genetic studies in some cases, and just as importantly, clinical findings. Emerging data suggest that the heavy chain class-switched cases may be more like a clonal chronic lymphoproliferative disorder. Conclusions The 2 subtypes of PCMZL create different diagnostic challenges and require the use of a multiparameter approach. Although very indolent, it is important to distinguish PCMZLs from reactive proliferations, because they frequently recur and may require antineoplastic therapies. It is also critical to distinguish PCMZLs from other B- or T-cell lymphomas so that patients are properly evaluated and not overtreated.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4407-4407
Author(s):  
Gregorio Ignacio ◽  
Francisco Tripp ◽  
Petty Rodríguez ◽  
Mario Martínez ◽  
Concepción Martínez ◽  
...  

Abstract Background: Among the NHL, B Cells NHL are more frequent than T Cells NHL (6%) as described in USA & European reports. The highest frequencies of T Cell NHL have been reported in Japan with 9% and India with 12%. In a Mexican retrospective study we found a T Cell NHL frequency of 13%. Objective: To corroborate the patterns and frequency of B and T cell lymphomas in Mexico. Methods: The registry of lymphoid neoplasms was created based on the WHO classification. The Lymphoma subtype analysis was prepared in two periods: retrospectively from 2002 to December 2005 and prospectively from January to December 2006. The data recorded were: age, sex, cell type (B o T), NHL frequency, primary site, stage and prognostic index (IPI and FLIPI). Results: In the first group 2375 Lymphomas were included: 2122 B Cell NHL (89.34%) and 253 T-cell lymphomas 253 (10.6%). B-cell NHL: gender 55.17% male and 44.66 female, median age of 55.21 years Age >60 years 45.7%; 62% III–IV stage. After applying the FLIPI index, the patients were divided into three risk groups: low (8.4% of cases), intermediate (81%), and high (10.6%). The distribution of patients in IPI risk groups was 15.7%, 76%, and 8.3% of cases classified as low, intermediate, and high risk The frequencies of B cell lymphoma were: 49% DLBCL, 15% Follicular Lymphoma, 5.35% CLL/SLL, 1.6% Mantle cell Lymphoma, 0.9% Follicular Center Lymphoma, 1.6% Marginal Zone B Lymphoma, 6.1% MALT, 1.6% Burkitt Lymphoma. T cell lymphomas were distributed in: Peripheral T Cell 253 (46.36%), Cutaneous Anaplastic 42 (16.09%), T/NKcell 35 (13.40%), Lymphoblastic 28 (6.16%), T non classifiable 10 (3.83%). The second group included 344 lymphomas; 309 (89.82%) B Cell NHL and 31 (10.01%) T cell Lymphomas. Gender 51.7 male and 48.3 female, medium age 57.79 years (SD 16.09); . >60 years 44%. After applying the FLIPI index the distribution of patients was 24.5% with intermedium risk and 9.5.8% high risk. Patients were divided into three IPI risk groups: Low 69.2% Intermediate 23% and high risk 7.8%. The frequencies of B cell lymphomas subtype were: DLBCL 168 (52.3%), Follicular 58(18.4%), CLL/SLL 19 (6.14%), Mantle Cell 10 (3.2%), Follicle Center Lymphoma (0.9%), Marginal Zone B 4 (1.2%), MALT 14 (4.5%), Burkitt’s Lymphoma 5 (1.6%). The T Cell Lymphoma subgroup frequencies were: T Cell Peripheral 7 (22.5%), Cutaneous Anaplastic 5 (16.1%), N/K cell 4 (12.9), Lymphoblastic 3 (9.6%), T lymphoma non classifiable 6 (19%). Conclusions: We confirmed a high incidence of T cell NHL in consecutive registries in Mexico. In the B cell subgroup it seems to be a difference where the DLBCL has a higher frequency and the CLL/SLL subgroup the lowest compared with other series. These differences in frequency might be explained by ethnic characteristics, however we need more epidemiological and viral studies, looking for Epstein Barr virus.


2020 ◽  
Author(s):  
Qingqing Pan ◽  
Yaping Luo ◽  
Yan Zhang ◽  
Long Chang ◽  
Ji Li ◽  
...  

Abstract Background: In order to study the CXCR4 expression with [68Ga]pentixafor PET in different types of non-Hodgkin lymphoma, we performed a retrospective study to describe the [68Ga]pentixafor PET/CT imaging in a spectrum of lymphomas and to compare it with [18F]FDG PET/CT. Results: Twenty-seven patients with newly diagnosed non-Hodgkin lymphoma were recruited retrospectively. [68Ga]pentixafor PET showed increased radioactivity in lymphoplasmacytic lymphoma (n = 8), marginal zone lymphoma (n = 4), diffuse large B cell lymphoma (n = 3), follicular lymphoma (n = 2), mantle cell lymphoma (n = 1), unclassified indolent B cell lymphoma (n = 3) and enteropathy associated T cell lymphoma (n = 3). However, peripheral T cell lymphoma, not otherwise specified (n = 1), and NK/T cell lymphoma (n = 2) were not avid for [68Ga]pentixafor. In comparison to [18F]FDG PET, [68Ga]pentixafor PET demonstrated more extensive disease and higher radioactivity in lymphoplasmacytic lymphoma and marginal zone lymphoma. Conclusion: CXCR4 expression varies in different types of non-Hodgkin lymphoma. Overexpression of CXCR4 was detected with [68Ga]pentixafor PET/CT in lymphoplasmacytic lymphoma, marginal zone lymphoma, diffuse large B cell lymphoma, follicular lymphoma, mantle cell lymphoma, unclassified indolent B cell lymphoma, and enteropathy associated T cell lymphoma. The uptake of [68Ga]pentixafor was higher than [18F]FDG in lymphoplasmacytic lymphoma and marginal zone lymphoma.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2386-2386
Author(s):  
Marcelo Bellesso ◽  
Renato Centrone ◽  
Daniela Ferreira Dias ◽  
Rodrigo Santucci

Abstract Advanced disease means a poor prognosis factor in patients with non-Hodgkin Lymphoma (NHL). There are few Brazilian data about epidemiologic incidence of NHL, however it was observed that high grade B cell Lymphoma is more frequent than in North America, Argentina and Chile, besides that we have deficient information about Brazilian private service care. Such knowledge is essential to plan economic resources to promote the best treatment and diagnosis. Moreover, is fundamental to elaborate electronic data storage to promote a continuous supply flow of information during clinical practice. We have designed an accessible and automated data collection model built into the electronic medical records for patients with NHL. Here, we show some epidemiological parameters and classic risk factors about NHL in a private care institute stocked by continuous electronic data storage. We evaluated data from patients with NHL during November 2015 to May 2016. Information was obtained by decentralized automated model after each outpatient care. We studied 229 patients with NHL. It was observed 112(48.9%) patients with Diffuse Large B cell Lymphoma not otherwise specified (DLBCL), 65(28.3%) Follicular Lymphoma (FL), 21(9.1%) Mantle Cell Lymphoma (MCL), 12(5.2%) Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue gastric lymphoma (GMALT), 6(2.9%) Extranodal marginal zone lymphoma of nonGastric MALT lymphoma (non-GMALT), 4(1.7%) Splenic B-cell marginal zone lymphoma (SL), 1(0.4%) nodal marginal zone lymphoma (NMZL), 1(0.4%) Sporadic Burkitt Lymphoma (BL), 2(0.8%) peripheral T cell lymphoma not otherwise specified (PTCL), 2(0.8%) anaplastic large T Cell lymphoma, ALK negative (ALTCL), 2(0.8%) Extranodal NK/T-cell lymphoma, basal type and 1(0.4%) mycosis fungoides (MF). It was evidenced advanced disease stage III/IV in DLBCL, FL and MCL: 70(62.5%), 54 (87.1%), 18 (85.7%); Extranodal infiltration sites in DLBCL, FL, MCL: 35/94 (37.2%), 11/50 (22%) and 5/18( 27.7%); Elevated serum LDH in DLBCL, FL, MCL: 31/74( 43.6%), 10/38 (26.3%) and 4/14 (28.5%), Performance status ≥ 2 in DLBCL, FL, MCL: 18/75(24%), 2/28 (4.1) 12/20 (60%), respectively. In this study, we have demonstrated that decentralized electronic data storage was useful and could appear an attractive model for clinical practice; moreover, it was possible observed high incidence of DLCBL and FL with advanced disease. Disclosures No relevant conflicts of interest to declare.


2006 ◽  
Vol 30 (1) ◽  
pp. 128-132 ◽  
Author(s):  
Sa A Wang ◽  
Neal Olson ◽  
Lawrence Zukerberg ◽  
Nancy Lee Harris

Piel ◽  
2017 ◽  
Vol 32 (5) ◽  
pp. 314-316
Author(s):  
Carolina Areán ◽  
Alicia Córdoba ◽  
Juan García ◽  
Amaia Larumbe

2021 ◽  
Vol 6 (1) ◽  
Author(s):  
Condom M ◽  
◽  
Climent F ◽  
Varela M ◽  
Gonzalez Barca E ◽  
...  

Marginal Zone Lymphomas (MZL) and Peripheral T Cell Lymphomas (PTCL) are uncommon neoplasms and patients affected by both lymphomas in a lifetime have been rarely reported. Here we present two patients with an initial diagnosis of a MZL who further developed a PTCL. We also present the review of the literature of six other cases of MZL. Half of the cases reported are synchronous and the other half metachronous. Most MZLs reported are MALT subtype. There is no predominant subtype amongst PTCLs. Chronic exposure to proinflammatory cytokines, genetic predisposition or exposure to carcinogenic agents could be possible causes underlying this rare phenomenon.


Cancer ◽  
2007 ◽  
Vol 109 (6) ◽  
pp. 1146-1151 ◽  
Author(s):  
Naoto Tomita ◽  
Shigeki Motomura ◽  
Rie Hyo ◽  
Hirotaka Takasaki ◽  
Sachiya Takemura ◽  
...  

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1907-1907
Author(s):  
Mitchell R. Smith ◽  
Indira J. Joshi ◽  
Fang Jin ◽  
Tahseen Al-Saleem

Abstract Background: Mantle cell lymphoma (MCL) is characterized by t(11;14) which dysregulates cyclin D1 expression. Eμ-cyclinD1 transgenic mice, however, are healthy. Additional genetic events must be necessary for lymphomagenesis, and knowledge of these would enhance understanding and therapy of MCL. In addition, a mouse model of MCL would be helpful in drug development. Alterations in p53 have been described in MCL, often associated with the blastic variant. Objectives and Methods: To determine whether p53 and cyclin D1 can cooperate in lymphomagenesis, we cross bred Eμ-cyclinD1 transgenic mice (Bodrug et al EMBO J, 1996, courtesy of Alan Harris) with mice transgenic for mutant p53 (Jackson Labs, Jacks et al Curr Biol, 1994). Progeny mice were monitored for presence of the transgenes by PCR of tail vein DNA and observed for development of disease. Results: Of mice carrying both aberrant genes, 24 of 38 developed B cell lymphoma. Mice did not become visibly ill until at least 12 months of age, with median age at sacrifice 15.5 (range 12–23) months. The lymphoma was generally disseminated, involving spleen, liver, diffuse adenopathy and marrow with occasional extranodal sites. Histology varied between small and large cell, with some having a vaguely follicular growth pattern. T cell lymphomas occurred in 2 other mice, while 5 developed osteosarcoma (1 of these in a mouse that also had B cell lymphoma). The B cell lymphomas were clonal by Cμ-VH PCR. Cyclin D1 expression was documented by Western analysis. A cell line has also been developed from one of the B cell lymphomas and this line rapidly grows into disseminated lymphoma in syngeneic mice. These B cell lymphomas differ from the thymic T cell lymphomas seen in heterozygous p53 mutant mice that do not co-express cyclin D1. The latency period differs from cyclin D1 x myc double transgenic mice. Conclusions: This model demonstrates cooperation between p53 and cyclin D1 pathways in B cell lymphomagenesis and should prove useful in delineating how these signals interact. The cell line may prove useful in pre-clinical testing of new agents for MCL.


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