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International Journal of Radiation... May 2015Primary cutaneous lymphomas are a heterogeneous group of diseases. They often remain localized, and they generally have a more indolent course and a better prognosis...
Primary cutaneous lymphomas are a heterogeneous group of diseases. They often remain localized, and they generally have a more indolent course and a better prognosis than lymphomas in other locations. They are highly radiosensitive, and radiation therapy is an important part of the treatment, either as the sole treatment or as part of a multimodality approach. Radiation therapy of primary cutaneous lymphomas requires the use of special techniques that form the focus of these guidelines. The International Lymphoma Radiation Oncology Group has developed these guidelines after multinational meetings and analysis of available evidence. The guidelines represent an agreed consensus view of the International Lymphoma Radiation Oncology Group steering committee on the use of radiation therapy in primary cutaneous lymphomas in the modern era.
Topics: Consensus; Humans; Lymphoma, B-Cell; Lymphoma, B-Cell, Marginal Zone; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Large-Cell, Anaplastic; Lymphoma, Non-Hodgkin; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Patient Positioning; Prognosis; Radiation Tolerance; Radiotherapy Dosage; Skin Neoplasms
PubMed: 25863751
DOI: 10.1016/j.ijrobp.2015.01.008 -
Dermatologie (Heidelberg, Germany) Oct 2022Primary cutaneous lymphomas (CL) are highly radiosensitive. Therefore, radiotherapy is an integral part of multimodality treatment. (Review)
Review
BACKGROUND
Primary cutaneous lymphomas (CL) are highly radiosensitive. Therefore, radiotherapy is an integral part of multimodality treatment.
AIM
The present work provides an overview of indications, technical developments, and dose concepts for total skin electron beam therapy (TSEBT), local radiotherapy as well as maintenance therapy, and current combination studies regarding cutaneous T‑ and B‑cell lymphomas.
MATERIALS AND METHODS
We performed a selective literature search in the PubMed database on the topic of radiotherapy for CL and a search for current studies using clinicaltrials.gov. Furthermore, we describe our own treatment strategies and summarize national and international guidelines.
RESULTS
Low-dose TSEBT is nationally and internationally recommended as an alternative to conventional 36 Gy TSEBT. The main advantages are better tolerability, the possibility of retreatment, a shorter treatment course (approximately 3 weeks), and a short time to response. In current studies, TSEBT is usually delivered to a total dose of 12 Gy and combined with immunotherapy and epigenetic therapy. Local radiotherapy is indicated for mycosis fungoides (MF) tumors and is a curative treatment regimen for other CL, particularly primary cutaneous B‑cell lymphomas.
CONCLUSION
TSEBT is a very effective treatment for MF and is a highly effective palliative treatment, leading to rapid symptom relief and improvement in quality of life. It is an important treatment option, especially in patients with extensive generalized lesions or advanced tumor stage. Local radiation is used as part of TSEBT for tumors and as a boost to undertreated areas. Other CLs are primarily curable with local radiotherapy.
Topics: Humans; Lymphoma, B-Cell; Mycosis Fungoides; Quality of Life; Skin; Skin Neoplasms
PubMed: 36018330
DOI: 10.1007/s00105-022-05046-w -
Chinese Clinical Oncology Feb 2019Mycosis fungoides (MF) and Sézary syndrome (SS) are two well-characterized skin limited and leukemic subtypes of cutaneous T-cell lymphoma (CTCL), respectively.... (Review)
Review
Mycosis fungoides (MF) and Sézary syndrome (SS) are two well-characterized skin limited and leukemic subtypes of cutaneous T-cell lymphoma (CTCL), respectively. Progressive global immune dysfunction and a multitude of specific immunological abnormalities have long been recognized as features of MF and SS. Therefore, a variety of immune-based therapies have been explored and used in the clinic for decades in the attempt to restore the immune imbalance in these malignancies. With recent advances in the development of novel immunotherapies in cancer treatment, new treatment modalities have emerged to complement the existing repertoire. Herein, we provide a comprehensive review of immune evasive mechanisms in MF/SS and summarize the established and emerging immunotherapies for these malignancies. We explain the underlying mechanisms of these immune-based therapies and derive recommendation from results of major clinical trials.
Topics: Humans; Immunotherapy; Mycosis Fungoides; Sezary Syndrome
PubMed: 30691274
DOI: 10.21037/cco.2019.01.01 -
Dermatology Online Journal Dec 2018Folliculotropic mycosis fungoides (MF) is a distinct subset of cutaneous T cell lymphoma (CTCL). The disease is typically marked by an aggressive course and is often...
Folliculotropic mycosis fungoides (MF) is a distinct subset of cutaneous T cell lymphoma (CTCL). The disease is typically marked by an aggressive course and is often recalcitrant to skin-direct therapy. We report a case of an 83-year-old woman with folliculotropic MF characterized by erythematous, scaly plaques on the forehead along with poliosis and alopecia of the right medial eyebrow.
Topics: Aged, 80 and over; Alopecia; Eyebrows; Facial Neoplasms; Female; Humans; Mycosis Fungoides; Pigmentation Disorders; Skin Neoplasms
PubMed: 30677794
DOI: No ID Found -
Acta Dermato-venereologica Mar 2021Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. The inflammatory micro-environment in mycosis fungoides is complex. There is accumulating...
Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. The inflammatory micro-environment in mycosis fungoides is complex. There is accumulating evidence that the neoplastic T-cells take control of the microenvironment and thereby promote their own expansion by suppressing cellular immunity. B-cells have proved to be upregulated in large-cell transformed mycosis fungoides, and could potentially play a role in disease progression. To investigate the presence of B-cells in mycosis fungoides compared with controls, this study analysed 85 formalin-fixed and paraffin-embedded mycosis fungoides biopsies. MS4A1 gene expression was significantly upregulated in mycosis fungoides compared with controls (p < 0.0001) and further upregulated in disease progression, (p = 0.001). Digital quantification of PAX5+/CD20+ cells confirmed the increased presence of B-cells in mycosis fungoides compared with controls. No co-labelling of CD3/CD20 was observed in the neoplastic T-cells. This study found a significantly increased presence of B-cells in the tumour-associated microenvironment in mycosis fungoides. These findings could potentially lead to new treatment strategies for mycosis fungoides.
Topics: Antigens, CD20; B-Lymphocytes; Humans; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Skin Neoplasms; Tumor Microenvironment
PubMed: 33686443
DOI: 10.2340/00015555-3775 -
International Journal of Molecular... Nov 2021Mycosis fungoides (MF) and Sézary syndrome (SS), the most common types of cutaneous T-cell lymphoma (CTCL), are characterized by proliferation of mature CD4+ T-helper...
Mycosis fungoides (MF) and Sézary syndrome (SS), the most common types of cutaneous T-cell lymphoma (CTCL), are characterized by proliferation of mature CD4+ T-helper cells. Patients with advanced-stage MF and SS have poor prognosis, with 5-year survival rates of 52%. Although a variety of systemic therapies are currently available, there are no curative options for such patients except for stem cell transplantation, and thus the treatment of advanced MF and SS still remains challenging. Therefore, elucidation of the pathophysiology of MF/SS and development of medical treatments are desired. In this study, we focused on a molecule called OX40. We examined OX40 and OX40L expression and function using clinical samples of MF and SS and CTCL cell lines. OX40 and OX40L were co-expressed on tumor cells of MF and SS. OX40 and OX40L expression was increased and correlated with disease severity markers in MF/SS patients. Anti-OX40 antibody and anti-OX40L antibody suppressed the proliferation of CTCL cell lines both in vitro and in vivo. These results suggest that OX40-OX40L interactions could contribute to the proliferation of MF/SS tumor cells and that the disruption of OX40-OX40L interactions could become a new therapeutic strategy for the treatment of MF/SS.
Topics: Antibodies, Anti-Idiotypic; Antigens, Differentiation; Cell Line, Tumor; Cell Proliferation; Gene Expression Regulation, Neoplastic; Humans; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; OX40 Ligand; Sezary Syndrome
PubMed: 34830466
DOI: 10.3390/ijms222212576 -
European Journal of Cancer (Oxford,... Apr 2018Our current mycosis fungoides (MF) and Sézary Syndrome (SS) staging system includes blood-classification from B0-B2 for patch/plaque/tumour or erythroderma based on... (Review)
Review
Blood classification and blood response criteria in mycosis fungoides and Sézary syndrome using flow cytometry: recommendations from the EORTC cutaneous lymphoma task force.
Our current mycosis fungoides (MF) and Sézary Syndrome (SS) staging system includes blood-classification from B0-B2 for patch/plaque/tumour or erythroderma based on manual Sézary counts but results from our EORTC survey confirm these are rarely performed in patch/plaque/tumour MF, and there is a trend towards using flow cytometry to measure blood-class. Accurately assigning blood-class effects overall stage and the 'global response' used to measure treatment responses in MF/SS and hence impacts management. The EORTC Cutaneous Lymphoma Task Force Committee have reviewed the literature and held a Workshop (June 2017) to agree a definition of blood-class according to flow cytometry. No large study comparing blood-class as defined by Sézary count with flow cytometry has been performed in MF/SS. The definition of blood-class by flow cytometry varies between publications. Low-level blood involvement occurs in patch/plaque/tumour much less than erythroderma (p < 0.001). The prognostic relevance of blood involvement (B1 or B2) in patch/plaque/tumour is not known. Studies have not shown a statistically worse difference in prognosis in erythrodermic MF patients with low-level blood involvement (IIIB) versus those without (IIIA), but Sezary patients who by definition have a leukaemic blood picture (staged IVA1 or higher) have a worse prognosis. For consistency flow, definition for blood-class must be an objective measurement. We propose absolute counts of either CD4+CD7-or CD4+CD26-where B0<250/μL, B1 = 250/μl-<1000/μL and B2≥1000/μL plus a T-cell blood clone. Fluctuations between B0 and B1 should not be considered in the treatment response criteria until further prognostic information is known.
Topics: Flow Cytometry; Humans; Lymphoma; Mycosis Fungoides; Neoplasm Staging; Sezary Syndrome; Skin Neoplasms
PubMed: 29477101
DOI: 10.1016/j.ejca.2018.01.076 -
Advances in Therapy Sep 2022Mycosis fungoides (MF) is a rare disease and is the most common form of cutaneous T cell lymphoma. Topical chlormethine (CL) gel is the first cytotoxic chemotherapy gel... (Review)
Review
Mycosis fungoides (MF) is a rare disease and is the most common form of cutaneous T cell lymphoma. Topical chlormethine (CL) gel is the first cytotoxic chemotherapy gel that was specifically developed for treatment of MF. In this review, we provide an overview of all available data on the use of CL gel for treatment of patients with MF. On the basis of the current data collected, CL gel is highly effective, with good response rates observed both in clinical trial and real-world settings. While the gel is approved for monotherapy, it is also used in combination with concomitant skin-directed or systemic therapies in clinical practice. Responses to CL gel treatment can be rapid, but they also frequently occur with a delayed onset of up to 6 months. This indicates that continued treatment with CL gel is important. CL gel has a manageable safety profile, with most adverse events being mild and skin related. Contact dermatitis is one of the more common skin-related adverse events to occur with CL gel treatment that can potentially lead to treatment discontinuation. The data from the literature indicate that patients being treated with CL gel should be monitored carefully, and that dermatitis must be managed effectively to allow patients to continue treatment and achieve the best possible response to treatment.
Topics: Clinical Trials as Topic; Gels; Humans; Lymphoma, T-Cell, Cutaneous; Mechlorethamine; Mycosis Fungoides; Skin Neoplasms
PubMed: 35852707
DOI: 10.1007/s12325-022-02219-w -
Dermatologic Clinics Oct 2015Mycosis fungoides (MF) and Sézary syndrome (SS) are common types of primary cutaneous T-cell lymphoma. Early-stage MF has a favorable prognosis and responds well to... (Review)
Review
Mycosis fungoides (MF) and Sézary syndrome (SS) are common types of primary cutaneous T-cell lymphoma. Early-stage MF has a favorable prognosis and responds well to skin-directed regimens. Patients with advanced-stage MF, transformed MF, and SS are treated with combined systemic and skin-directed therapies. However, the disease is incurable with standard regimens, and frequent relapses are common. Owing to the lack of improvement in overall survival with standard regimens, hematopoietic stem cell transplant (HSCT) has been explored as a potential curative option. This article reviews the role of HSCT in MF/SS and discusses data regarding conditioning regimens, treatment-related complications, and outcomes.
Topics: Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Lymphocyte Transfusion; Mycosis Fungoides; Myeloablative Agonists; Sezary Syndrome; Skin Neoplasms; Transplantation Conditioning; Transplantation, Autologous; Transplantation, Homologous
PubMed: 26433851
DOI: 10.1016/j.det.2015.05.014 -
Journal of Comparative Effectiveness... Jul 2023This study assessed the cost-utility of mogamulizumab, a novel monoclonal antibody, versus established clinical management (ECM) in UK patients in previously treated...
This study assessed the cost-utility of mogamulizumab, a novel monoclonal antibody, versus established clinical management (ECM) in UK patients in previously treated advanced mycosis fungoides (MF)/Sézary syndrome (SS). Lifetime partitioned survival model based on overall survival, next treatment-free survival and the use of allogeneic stem cell transplant was developed. Inputs were from the pivotal MAVORIC trial, real-world evidence and published literature. Extensive sensitivity analyses were conducted. Discounted incremental quality-adjusted life years (QALYs), costs and incremental cost-effectiveness ratio were 3.08, £86,998 and £28,233. Results were most sensitive to the survival extrapolations, utilities and costs after loss of disease control. Mogamulizumab is a cost-effective alternative to ECM in UK patients with previously treated advanced MF/SS.
Topics: Humans; Sezary Syndrome; Cost-Benefit Analysis; Skin Neoplasms; Mycosis Fungoides; Lymphoma, T-Cell, Cutaneous
PubMed: 37338181
DOI: 10.57264/cer-2023-0028