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Cutaneous Lymphoma
Published in Debjani Sahni, Adam Lerner, Bilal Fawaz, Advanced Skin Cancer, 2022
MF represents the most common form of CTCL overall, with an annual incidence of 0.4/100,000 in the United States.3 This generally indolent lymphoma is characterized by a protracted evolution of disease stage from patch to plaque, and eventually to tumor stage, often requiring years to progress between the stages. Additionally, not all patients progress to more advanced stages, and may remain, for example, as patch stage for the rest of their life.5,6 Diagnosis is often delayed due to the considerable variability in clinical presentation, often mimicking benign skin disorders, and because non-specific histologic findings are typically seen early in the disease process.5
Non-Melanoma Skin Cancer
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Irene De Francesco, Sean Whittaker, Stephen L. Morris
Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is considered part of the broad spectrum of extranodal marginal zone B-cell lymphomas commonly involving mucosal sites, the so-called mucosal associated lymphoid tissue (MALT). It is an indolent lymphoma composed of small cells including marginal zone cells, lymphoplasmacytoid cells, and plasma cells. It includes cases previously designated as primary cutaneous immunocytoma and exceptional cases of primary cutaneous plasmacytoma without multiple myeloma.
Monoclonal Antibodies in the Treatment of Malignant Lymphomas and Chronic Lymphocytic Leukemia
Published in Gertjan J. L. Kaspers, Bertrand Coiffier, Michael C. Heinrich, Elihu Estey, Innovative Leukemia and Lymphoma Therapy, 2019
In the initial phase I/II study, 90Y-ibritumomab was administered on 51 patients with relapsed and refractory CD20-positive B-cell NHL (58). The overall response rate (ORR) for the 34 patients with indolent lymphoma was 82% (CR 26% and PR 56%). The estimate median TTP for the entire group was 12.9 plus months and the median duration of response was 11.7 plus months. The major toxicity of 90Y-ibritumomab was myelosuppression with thrombocytopenia being the most common. 90Y-ibritumomab has been compared with rituximab in a randomized controlled phase III study (59). The ORR was 80% (CR/CRu 34% and PR 45%) for 90Y-ibritumomab as compared with 56% (CR/CRu 20% and PR 36%) for rituximab (P = 0.002). The estimated TTP was 12.6 months for 90Y-ibritumomab and 10.2 months for rituximab (P = 0.062). In another study, Witzig treated with 90Y-ibritumomab 54 patients with FL refractory to rituximab (60). The ORR for the entire cohort was 74% (CR 14% and PR 59%). The estimated TTP and response duration for responders were 8.7 and 6.4 months, respectively. Long-term responses are seen in 37% of the patients with a median duration of response of 28 months in these good responders (61).
Lymphomas of the salivary glands: a systematic review
Published in Acta Oto-Laryngologica, 2023
Ahmed Ehsan Al-Khafaf, Fahd Al-Shahrestani, Yusuf Baysal, Lise Mette Rahbek Gjerdrum, Steffen Heegaard, Lars Møller Pedersen, Preben Homøe
This review shows that 33% of the patients had a history of Sjögren’s syndrome which is higher than the 20% that Kojima et al. have reported [15]. The high frequency of Sjögren’s syndrome in our review could be a result of publication bias. Although both EMZL and FL are regarded as indolent lymphomas, only EMZL is considered to have a strong association with Sjögren’s syndrome [7]. In fact, another study by Kojima et al. stated that FL is usually found in patients without Sjögren’s syndrome [19]. Our results support this difference, with 50% of the EMZL patients having a history of Sjögren’s syndrome while only 4% of the FL patients had a history of Sjögren’s syndrome. There is an indication of a higher stage and a 2.5-fold higher mortality in FL patients compared to EMZL patients at 12 months follow-up. This is similar to findings of earlier studies [20], although other studies have found the same prognosis for FL and EMZL [19]. In general, the majority of FL patients usually present with an advanced disease stage, unlike patients with EMZL. This may explain a difference in the long-term outcome between different types of indolent lymphoma.
How can we assess and measure prognosis for MALT lymphoma? A review of current findings and strategies
Published in Expert Review of Hematology, 2021
Barbara Kiesewetter, Markus Raderer
Regarding general prognosis, MALT lymphoma is classified as an indolent lymphoma with 5- and 10-year survival rates of 80 and 90%, nearly reaching the estimate of an age-matched general population [1]. However, cautious observation of the disease’s individual dynamics represents an integral part of patient management as MALT lymphoma may cause organ-related problems due to tumor progression or affect quality of life in case of symptoms, both commonly indicating the need for anti-tumor therapy. In addition, approximately 5% of patients experience transformation to aggressive diffuse large B-cell lymphoma (DLBCL), which results in crucial worsening of prognosis and need for aggressive therapy [7–10]. Current therapeutic options for MALT lymphoma patients include antibiotic eradication of H. pylori for gastric MALT lymphoma, local treatment with radiotherapy for non-disseminated disease and systemic treatment with chemotherapy ± rituximab (R) for symptomatic progressive disease [11]. Several cytostatic regimens adapted from other lymphoma entities have been evaluated, ranging from alkylating compounds to purine analogues and CHOP-based approaches, with R-chlorambucil and R-bendamustine currently constituting the most commonly applied treatments [12–14]. Recent phase II trials have also assessed the value of immunomodulatory strategies such as IMiDs or anti-CD20-antibody monotherapy, which might be of increasing importance in upcoming years, not at least in view of data in other indolent lymphoma entities such as follicular lymphoma [15].
Chemokine receptor CXCR4: An important player affecting the molecular-targeted drugs commonly used in hematological malignancies
Published in Expert Review of Hematology, 2020
Liangliang Li, Ye Chai, ChongYang Wu, Li Zhao
Mantle cell lymphoma (MCL) is a relatively uncommon heterogeneous B-cell lymphoma originating from the lymph node mantle zone, constituting approximately 5% of all B-cell lymphomas, and it has a broad spectrum of clinical behavior from indolent to highly aggressive cases. A small proportion of patients may present with clinical characteristics of indolent lymphoma. However, for most patients, extranodal invasion in peripheral blood, lymph nodes, and bone marrow is common. Traditional treatment methods are limited and the prognosis is poor [20]. Currently applied molecular-targeted drugs such as CD20 monoclonal antibodies, proteasome inhibitors, and BTK inhibitors are candidates for further improved treatment in MCL. Among these, the proteasome inhibitor Bz was initially approved for use in relapsed MCL. In the LYM-3002 trial in 2015, a Bz-based chemotherapy (Bz, rituximab, cyclophosphamide, doxorubicin, and prednisone) regimen showed significantly progression-free survival compared with the R-CHOP regimen in MCL, so from then on, Bz was licensed for first-line use in MCL [21,22].