-
Hematology/oncology Clinics of North... Feb 2019Cutaneous T-cell lymphomas are a heterogeneous collection of non-Hodgkin lymphomas that arise from skin-tropic memory T lymphocytes. Among them, mycosis fungoides (MF)... (Review)
Review
Cutaneous T-cell lymphomas are a heterogeneous collection of non-Hodgkin lymphomas that arise from skin-tropic memory T lymphocytes. Among them, mycosis fungoides (MF) and Sézary syndrome (SS) are the most common malignancies. Diagnosis requires the combination of clinical, pathologic, and molecular features. Significant advances have been made in understanding the genetic and epigenetic aberrations in SS and to some extent in MF. Several prognostic factors have been identified. The goal of treatment is to minimize morbidity and limit disease progression. However, hematopoietic stem cell transplantation, considered for patients with advanced stages, is the only therapy with curative intent.
Topics: Biopsy; Combined Modality Therapy; Disease Management; Disease Susceptibility; Humans; Incidence; Mycosis Fungoides; Neoplasm Staging; Phenotype; Prognosis; Sezary Syndrome; Skin; T-Lymphocytes; Treatment Outcome
PubMed: 30497668
DOI: 10.1016/j.hoc.2018.09.001 -
Actas Dermo-sifiliograficas Apr 2017Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma. The clinical course of the disease is typically characterized by progression from a... (Review)
Review
Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma. The clinical course of the disease is typically characterized by progression from a nonspecific phase of erythematous macules to the appearance of plaques and ultimately, in some patients, tumors. However, numerous clinical and histopathologic variants of MF with specific therapeutic and prognostic implications have been described in recent decades. Clarification of the differential diagnosis can be frustrated by the wide range of clinical manifestations and histopathologic patterns of cutaneous infiltration, particularly in the early phases of the disease. In this paper, we review the main clinical, histopathologic, and immunohistochemical characteristics of the variants of MF described in the literature in order to facilitate early diagnosis of the disease.
Topics: Humans; Mycosis Fungoides
PubMed: 27871620
DOI: 10.1016/j.ad.2016.08.009 -
Clinics in Dermatology 2019Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, typically presents in its early stage as inflammatory erythematous patches or plaques, with... (Review)
Review
Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, typically presents in its early stage as inflammatory erythematous patches or plaques, with epidermotropism as the histopathologic hallmark of the disease. Over the past 30 years, numerous atypical types of MF, which deviate from the classic Alibert-Bazin presentation of the disease, have been described. These variants can simulate a wide variety of benign inflammatory skin disorders either clinically, both clinically and histopathologically, or mainly histopathologically. We have summarized the many faces of the disease, which set MF as a "great imitator," with special focus on the differential diagnosis and its benign mimickers.
Topics: Diagnosis, Differential; Female; Humans; Male; Mycosis Fungoides; Skin
PubMed: 31178107
DOI: 10.1016/j.clindermatol.2019.01.004 -
Blood Sep 2007The ISCL/EORTC recommends revisions to the Mycosis Fungoides Cooperative Group classification and staging system for cutaneous T-cell lymphoma (CTCL). These revisions... (Review)
Review
Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC).
The ISCL/EORTC recommends revisions to the Mycosis Fungoides Cooperative Group classification and staging system for cutaneous T-cell lymphoma (CTCL). These revisions are made to incorporate advances related to tumor cell biology and diagnostic techniques as pertains to mycosis fungoides (MF) and Sézary syndrome (SS) since the 1979 publication of the original guidelines, to clarify certain variables that currently impede effective interinstitution and interinvestigator communication and/or the development of standardized clinical trials in MF and SS, and to provide a platform for tracking other variables of potential prognostic significance. Moreover, given the difference in prognosis and clinical characteristics of the non-MF/non-SS subtypes of cutaneous lymphoma, this revision pertains specifically to MF and SS. The evidence supporting the revisions is discussed as well as recommendations for evaluation and staging procedures based on these revisions.
Topics: Europe; Humans; Mycosis Fungoides; Neoplasm Staging; Sezary Syndrome; Skin Neoplasms; Societies, Medical
PubMed: 17540844
DOI: 10.1182/blood-2007-03-055749 -
Current Treatment Options in Oncology Jan 2021While most patients with early-stage mycosis fungoides (MF) follow an indolent course, patients with advanced-stage MF/Sézary syndrome (SS) have a poor prognosis with a... (Review)
Review
While most patients with early-stage mycosis fungoides (MF) follow an indolent course, patients with advanced-stage MF/Sézary syndrome (SS) have a poor prognosis with a median survival of less than 5 years. Although there are a number of treatments currently available, achieving and maintaining a durable response remain challenging, especially in advanced-stage MF/SS. The choice of frontline therapy is dependent on the stage of disease. For early-stage MF, the treatment concept is to control skin lesions mainly by skin-directed therapies, such as topical therapies, phototherapies, and radiotherapies. For advanced-stage MF/SS, systemic treatments by biological or targeted therapies including bexarotene and interferon either alone or in combination are tried first, with more immunosuppressive chemotherapies being reserved for refractory or rapidly progressive disease. Recent improvements in biological or targeted therapies include brentuximab vedotin and mogamulizumab. When biopsy samples have 10% or more CD30-positive malignant cells, brentuximab vedotin, an anti-CD30 antibody conjugated to monomethyl auristin E, can be a desirable treatment option. For cases with blood involvement, mogamulizumab, an antibody binding to C-C chemokine receptor 4, is effective with high response rates. In the refractory setting, alemtuzumab, histone deacetylase inhibitors, pralatrexate, gemcitabine, and doxorubicin are considered as the treatment option. Because only allogeneic hematopoietic stem cell transplantation can offer a chance of cure with durable complete remission, advanced-stage patients with a markedly short life expectancy should be evaluated for eligibility. Given that there are few randomized controlled studies in the literature, it is necessary to investigate which therapy is preferable for each patient with MF/SS by comparative prospective trials.
Topics: Clinical Decision-Making; Combined Modality Therapy; Disease Management; Disease Progression; Humans; Mycosis Fungoides; Neoplasm Grading; Neoplasm Staging; Prognosis; Sezary Syndrome; Skin Neoplasms; Treatment Outcome
PubMed: 33415447
DOI: 10.1007/s11864-020-00809-w -
Journal of Cosmetic Dermatology Jul 2022Ten-year survival rates in mycosis fungoides (MF) broadly varies, however, there is no standardized prognostic index available. This is presumably due to low prevalence,... (Review)
Review
BACKGROUND
Ten-year survival rates in mycosis fungoides (MF) broadly varies, however, there is no standardized prognostic index available. This is presumably due to low prevalence, heterogeneity, and diagnostic challenges in MF. Recent studies have focused on identifying objective prognostic indices by using different parameters for survival determinants. The Cutaneous Lymphoma International Prognostic Index (CLIPI) and the Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) represent prototypical studies that identify prognostic factors, seeking to improve management and outcomes in early-stage MF. Detecting these factors and stratifying MF patients according to their disease progression risk may help to manage these patients more efficiently.
AIMS
Review the current literature to determine the risk factors determining prognosis in MF.
METHODOLOGY
A Comprehensive literature search was performed using electronic online databases "PubMed" and "Google Scholar" using key words 'prognostic factor', 'prognostic indicator', 'mycosis fungoides', 'Sezary syndrome', 'Skin Lymphoma', 'Cutaneous Lymphoma'. Articles published in English language were considered for the review.
RESULTS
The strongest prognostic factor in MF patients is the stage of the disease. T stage and the presence of extracutaneous disease are the most important factors for survival. Other factors that are associated with worse prognosis are male gender, age >60, presence of plaques, folliculotropism, eosinophilia and lymph node stage above N1/Nx. Elevated LDH was associated with later tumor stages and large cell phenotype at diagnosis had a better prognosis. KIR3DL2 was associated with malignant transformation.
CONCLUSION
The PROCLIPI study has assessed risk factors collected in MF patients from different countries and across different ethnicities following a rigorous clinicopathologic process. The findings presented here illustrated that disease prognosis in early stages depends on many contributing factors. Detection and stratification of such factors may allow a personalized approach to management of these patients.
Topics: Female; Humans; Male; Mycosis Fungoides; Neoplasm Staging; Prognosis; Prospective Studies; Skin Neoplasms
PubMed: 34687485
DOI: 10.1111/jocd.14528 -
Giornale Italiano Di Dermatologia E... Apr 2017Cutaneous T-cell lymphoma (CTCL) is a rare but increasing malignancy whose protean manifestations necessarily present in the integument, but can also spread to involve... (Review)
Review
Cutaneous T-cell lymphoma (CTCL) is a rare but increasing malignancy whose protean manifestations necessarily present in the integument, but can also spread to involve blood, lymph nodes and internal organs. We have developed efficacious and varied therapies to treat early and advanced stage disease, but there are still many who suffer tremendously from this illness. Although the pathogenesis of this cancer remains frustratingly elusive, over the last 200 years we have generated a robust body of evidence that points toward possible singular as well as multifactorial etiologies. Combining the historical hypotheses which have focused upon the concept of infectious causes, including carcinogenic genomic viral integration and bacterial superantigenic chronic stimulation as well as industrial/occupational exposure, along with the more recent revelations of both genetic and epigenetic alteration and immune dysregulation, we are closer than ever to understanding the etiology of CTCL. It is through this knowledge and continued research efforts that we will be able to better diagnose, treat, and potentially prevent or cure CTCL.
Topics: Epigenesis, Genetic; Humans; Lymphatic Metastasis; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Neoplasm Staging; Skin Neoplasms
PubMed: 27982549
DOI: 10.23736/S0392-0488.16.05527-9 -
Hematological Oncology Jun 2021Cutaneous T-cell lymphomas (CTCL) represent the majority of primary cutaneous lymphomas (CL). Mycosis fungoides (MF) and cutaneous CD30+ lymphoproliferative disorders... (Review)
Review
Cutaneous T-cell lymphomas (CTCL) represent the majority of primary cutaneous lymphomas (CL). Mycosis fungoides (MF) and cutaneous CD30+ lymphoproliferative disorders account for 80% of all CTCL. CTCL show overlapping histological features. Thus clinical-pathological correlation is of importance to achieve final diagnosis. MF shows a characteristic evolution with patches, plaques, and in a subset of patients (10%-20%) with tumors. Therapy is stage-adapted with skin-directed therapies such as UV-light therapies and corticosteroids in early disease stage (i.e., patch and limited plaque stage) and systemic therapies (retinoids, interferon, mono chemotherapy, targeted therapy) and/or radiation therapy (local or total skin beam electron) in advanced stages. Novel therapies include targeted therapy such as mogamulizumab (anti-CCR4) or brentuximab vedotin (anti-CD30) and histone deacetylase inhibitors. Considering the impact of targeted therapies, biomarkers such as CD30 are not only crucial for the diagnosis and correct classification of an individual lymphoma case, but also for therapy as they may represent therapeutic targets. In the recently revised WHO classification 2017 and the updated WHO-EORTC classification for CL 2018, primary cutaneous CD8+ acral T-cell lymphoma has been introduced as a new still provisional entity. It displays characteristic clinical, histological, and phenotypic features and exhibits an excellent prognosis. Rare, but aggressive CTCL include cutaneous primary cutaneous aggressive epidermotropic CD8-positive T-cell lymphoma and cutaneous gamma/delta T-cell lymphoma, which present with rapid onset of necrotic or ulcerated plaques and tumors. As they have a poor prognosis, treatment includes multiagent chemotherapy and hematopoietic stem cell transplantation.
Topics: Humans; Mycosis Fungoides; Skin Neoplasms
PubMed: 34105822
DOI: 10.1002/hon.2850 -
Journal of the American Academy of... May 2023
Topics: Humans; Sezary Syndrome; Mycosis Fungoides; Antibodies, Monoclonal, Humanized; Skin Neoplasms
PubMed: 36481378
DOI: 10.1016/j.jaad.2022.12.001 -
Hematology/oncology Clinics of North... Apr 2017Mycosis fungoides and the Sezary syndrome (SS) are rare lymphomas of CD4 helper T cells. There is stagewise progression from patch/plaques to thicker tumor... (Review)
Review
Mycosis fungoides and the Sezary syndrome (SS) are rare lymphomas of CD4 helper T cells. There is stagewise progression from patch/plaques to thicker tumor lesions/diffuse erythroderma. Blood involvement is a characteristic of SS. Outcomes are related to the extent of skin, blood, lymph node, and visceral organ involvement. Patients with limited patch and plaque disease are treated with skin-directed therapies. More advanced/refractory disease is treated with skin-directed therapies and oral or systemic immunomodulatory agents. Single-agent chemotherapies are used against tumors, refractory plaques, and lymph node and visceral involvement. Allogeneic stem cell transplantation is a potentially curative strategy for advanced/resistant disease.
Topics: Allografts; Humans; Immunologic Factors; Mycosis Fungoides; Sezary Syndrome; Stem Cell Transplantation; T-Lymphocytes, Regulatory
PubMed: 28340880
DOI: 10.1016/j.hoc.2016.11.008