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Principles of the WHO classification with special reference to aggressive extranodal lymphomas
Published in Franco Cavalli, Harald Stein, Emanuele Zucca, Extranodal Lymphomas, 2008
Harald Stein, Michael Hummel, Lorenz Trümper, Nancy Lee Harris
In addition to these common features between nodal and extranodal DLBCL, there are further molecular genetic characteristics which are typical for certain anatomical sites. Primary mediastinal large B-cell lymphoma is distinguished by gains on 9p21 and 2p14 as well as overexpression of JAK2.29,34 In DLBCL of the gastrointestinal tract, deletions of chromosome 7q22 and gains of 5, 15, and 16 are more frequent than in nodal DLBCL.35 Translocation (3;14) involving FoxP1 is almost exclusively found in gastric DLBCL.36 Primary DLBCL of the CNS are frequently associated with losses of 6p21 (HLA locus), which are also detectable in DLBCL occurring in the testis.37 Primary cutaneous DLBCL, leg type, which are usually of the ABC type, in many cases display additional amplifications of 18q21, affecting BCL2 and MALT1.38
Treatment strategies and survival outcomes of primary mediastinal large B-cell lymphoma
Published in Hematology, 2023
Lin Quan, Zhen He, Xiaoling Zuo, Lei Cao, Yi Wang, Hongyu Dai, Wei Wu, Xiao Shi, Hailing Liu
Primary mediastinal large B-cell lymphoma (PMBCL) is a rare and aggressive lymphoma that originates from the thymic and accounts for approximately 2% to 4% of all non-Hodgkin lymphomas [1,2]. Although it shares a similar histology with diffuse large B-cell lymphoma (DLBCL) and a genetic profile with classic Hodgkin lymphoma, PMBCL is recognized as a distinct entity in the 2016 World Health Organization classification [3,4]. Compared to DLBCL, PMBCL has a substantially better prognosis, with a 5-year survival rate of around 85% [5]. However, the most optimal therapy for PMBCL has yet to be determined, given the current lack of long-term follow-up data and prospective head-to-head trials [6]. An anthracycline-based polychemotherapy regimen combined with a CD20 monoclonal antibody is the backbone of PMBCL treatment at present [7,8]. There is limited evidence available on the role of consolidation radiotherapy in the modern era of PMBCL treatment, which has led to controversy surrounding its efficacy and safety. Given the recent release of the Surveillance, Epidemiology, and End Results (SEER) database with extended follow-up and broader population coverage, we conducted an evaluation of the latest survival rates and treatment strategies for PMBCL.
Risk assessment of venous thromboembolism in hematological cancer patients: a review
Published in Expert Review of Hematology, 2020
Thomas Sau-Yan Chan, Yu Yan Hwang, Eric Tse
In lymphoma, there is a remarkable variation in VTE incidence depending on the specific subtypes of lymphomas. Primary central nervous system lymphoma (PCNSL) is associated with the highest risk of thrombotic events, in the range of 40%–60% [30,31], followed by primary mediastinal large B-cell lymphoma [32]. In a meta-analysis involving 18,018 patients from 29 independent cohorts [31], the rate of thrombotic complication was 6.4%. Although this figure included patients with both arterial and venous thrombosis, majority of the events (5.3% out of 6.4%) were venous in nature. Furthermore, the risk of thrombosis was significantly higher in non-Hodgkin lymphoma (NHL) as compared with Hodgkin lymphoma (6.5% vs. 4.7%). Aggressive NHL or advanced-stage disease also had a higher incidence of events as compared with indolent NHL or early stage disease. Although over 95% of the events occurred during the treatment period, it was unclear if the development of thrombosis was primarily related to the therapies per se.
A positive approach: advances in proton therapy for the treatment of mediastinal lymphoma
Published in Expert Review of Hematology, 2020
Raymond B. Mailhot Vega, Bradford S. Hoppe
Regarding histologic subtypes of mediastinal lymphoma best treated with proton therapy, the modality has been employed in Hodgkin lymphoma, primary mediastinal large B-cell lymphoma, and diffuse large B-cell lymphoma [21]. In the absence of published experiences, one would posit a similar benefit in those with mediastinal Gray zone lymphoma. Others for whom proton therapy may be most beneficial include patients with relapsed or refractory disease. Indeed, in any patient who receives a significant amount of treatment and for whom the therapeutic ratio is highly sensitive, the reductions in cardiac and pulmonary dose achievable with proton therapy are all the more meaningful [22]. Despite these dosimetric benefits, proton therapy presents a few challenges. While phase II dosimetric evidence exists, there is no prospective level I evidence demonstrating the clinical superiority of protons, which, in turn, influences insurance coverage, limiting access to those patients for whom proton therapy may be most beneficial. Similarly, given the cost of facility construction, proton therapy centers represent fewer than 1% of radiotherapy of facilities, limiting access to those patients residing near the few existing centers, which are overwhelmingly located in populated urban areas. Finally, mediastinal lymphoma represents a diverse spectrum of disease presentations; therefore, individual anatomic distribution of disease portends the greatest potential benefit.