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Seminars in Cutaneous Medicine and... Mar 2018Mycosis fungoides (MF) is the most common type of cutaneous lymphoma. The term MF should be used only for the classical presentation of the disease characterized by the... (Review)
Review
Mycosis fungoides (MF) is the most common type of cutaneous lymphoma. The term MF should be used only for the classical presentation of the disease characterized by the evolution of patches, plaques, and tumors or for variants showing a similar clinical course. MF is divided into 3 clinical phases: patch, plaque, and tumor stage, and the clinical course is usually protracted over years or decades. Histopathologically, MF is characterized by an epidermotropic infiltrate of T lymphocytes that displays in most cases a helper phenotype. Cytotoxic variants are well described and do not have specific clinical, histopathological, or prognostic features. MF should be differentiated from other cutaneous epidermotropic lymphomas and from many inflammatory dermatoses with some similar clinicopathological features. The therapy of MF is planned mainly according to the stage and extent of the disease. In early phases, nonaggressive options represent the first-line strategy (eg, local corticosteroids, psoralen, and ultraviolet A [UV-A] irradiation, etc.). In patients with advanced disease, good results with potential for cure have been obtained with allogeneic stem cell transplantation, but toxicity is a serious limiting factor for this treatment. Conventional systemic chemotherapy and single-agent chemotherapy (eg, gemcitabine) give usually good results in advanced MF, but recurrences are the rule. Monoclonal antibodies directed against cluster of differentiation (CD)52 (alemtuzumab), CD30 (brentuximab vedotin), and chemokine receptor 4 (CCR4; mogamulizumab), as well as several other experimental therapies, have shown promising results and represent a valid alternative.
Topics: Humans; Mycosis Fungoides; Skin Neoplasms
PubMed: 29719014
DOI: 10.12788/j.sder.2018.002 -
Frontiers in Immunology 2023Discriminating between cutaneous anaplastic large cell lymphoma (cALCL) and CD30-positive transformed mycosis fungoides (CD30+ TMF) is challenging, particularly when...
BACKGROUND
Discriminating between cutaneous anaplastic large cell lymphoma (cALCL) and CD30-positive transformed mycosis fungoides (CD30+ TMF) is challenging, particularly when they arise in the context of pre-existing mycosis fungoides. The development of molecular diagnostic tools was hampered by the rarity of both diseases and the limited understanding of their pathogenesis.
METHODS
In this study, we established a cohort comprising 25 cALCL cases and 25 CD30+ TMF cases, with transcriptomic data obtained from 31 samples. We compared the clinicopathological information and investigated the gene expression profiling between these two entities. Furthermore, we developed an immunohistochemistry (IHC) algorithm to differentiate these two entities clinically.
RESULTS
Our investigation revealed distinct clinicopathological features and unique gene expression programs associated with cALCL and CD30+ TMF. cALCL and CD30+ TMF displayed marked differences in gene expression patterns. Notably, CD30+ TMF demonstrated enrichment of T cell receptor signaling pathways and an exhausted T cell phenotype, accompanied by infiltration of B cells, dendritic cells, and neurons. In contrast, cALCL cells expressed high levels of HLA class II genes, polarized towards a Th17 phenotype, and exhibited neutrophil infiltration. An IHC algorithm with BATF3 and TCF7 staining emerged as potential diagnostic markers for identifying these two entities.
CONCLUSIONS
Our findings provide valuable insights into the differential molecular signatures associated with cALCL and CD30+ TMF, which contribute to their distinct clinicopathological behaviors. An appropriate IHC algorithm could be used as a potential diagnostic tool.
Topics: Humans; Lymphoma, Large-Cell, Anaplastic; Ki-1 Antigen; Skin Neoplasms; Mycosis Fungoides; Gene Expression Profiling
PubMed: 37790936
DOI: 10.3389/fimmu.2023.1270365 -
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 -
Experimental Dermatology Aug 2017The practice of pre-emptive individualized medicine is predicated on the discovery, development and application of biomarkers in specific clinical settings. Mycosis... (Review)
Review
The practice of pre-emptive individualized medicine is predicated on the discovery, development and application of biomarkers in specific clinical settings. Mycosis fungoides and Sézary syndrome are the two most common type of cutaneous T-cell lymphoma, yet diagnosis, prognosis and disease monitoring remain a challenge. In this review, we discuss the current state of biomarker discovery in mycosis fungoides and Sézary syndrome, highlighting the most promising molecules in different compartments. Further, we emphasize the need for continued multicentre efforts to validate available and new biomarkers and to develop prospective combinatorial panels of already discovered molecules.
Topics: Biomarkers; Gene Expression; Humans; Mycosis Fungoides; Sezary Syndrome
PubMed: 27897325
DOI: 10.1111/exd.13261 -
The Australasian Journal of Dermatology Aug 2016Mycosis fungoides and Sézary syndrome are the most common variants of the cutaneous T-cell lymphomas. Assessment of a patient with a suspected diagnosis requires... (Review)
Review
Mycosis fungoides and Sézary syndrome are the most common variants of the cutaneous T-cell lymphomas. Assessment of a patient with a suspected diagnosis requires thorough history taking and physical examination, in combination with skin biopsy. In some cases flow cytometry, molecular studies and imaging are also required in order to diagnose and stage the disease. Staging is derived from the tumour-node-metastasis-blood classification and is currently our best attempt to stratify prognosis and hence guide management in this complex disease. Many other clinical, biological and pathological factors may help to distinguish groups at risk and predict prognosis more accurately. Management remains heavily guided by staging, such that patients with early-stage disease generally begin treatment with skin-directed or local therapies and those with advanced-stage disease have many treatment options, including chemotherapy, the use of biological agents, local and total body radiotherapy, as well as haematopoietic stem cell transplantation. Besides staging, many other patient-related factors influence the treatment strategy, particularly where symptom relief is paramount. There are many challenges remaining in the study of Mycosis fungoides and Sézary syndrome and, given the rarity of the disease, concerted worldwide efforts are required to conduct efficient and effective research.
Topics: Adult; Biological Products; Biomarkers, Tumor; Chemoradiotherapy; Combined Modality Therapy; Female; Hematopoietic Stem Cell Transplantation; Humans; Male; Middle Aged; Mycosis Fungoides; Neoplasm Invasiveness; Neoplasm Staging; Prognosis; Risk Assessment; Sezary Syndrome; Skin Neoplasms; Survival Analysis
PubMed: 25988337
DOI: 10.1111/ajd.12349 -
The American Journal of Dermatopathology Feb 2021Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T-cell lymphoma that has clinical overlap with a variety of inflammatory follicular unit disorders....
BACKGROUND
Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T-cell lymphoma that has clinical overlap with a variety of inflammatory follicular unit disorders. However, we describe distinctive presentations of FMF with acneiform features that can be diagnostically challenging, leading to diagnostic delay.
OBJECTIVE
To highlight the importance of histopathologic and immunohistochemical evaluation for diagnostic confirmation of presumed inflammatory follicular unit-based disorders that are unusual in presentation or unresponsive to standard therapies.
METHODS
A cross-sectional retrospective study of 5 consecutive patients with a histopathologic diagnosis of FMF was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases are presented.
RESULTS
We describe 5 patients with clinical and histopathologic presentations of FMF masquerading as hidradenitis suppurativa, furunculosis, or acne vulgaris (age range 34-66 years, 4:1 female to male). Clinical morphologies included open and closed comedones, inflammatory pustules, papules and nodules, follicular papules with keratotic plugging, cysts, and scarring involving the face, trunk, and intertriginous areas. All patients failed to respond to standard therapies, including topical and oral antibiotics, topical and oral retinoids, or topical corticosteroids, before receiving the diagnosis of FMF. Lesional skin biopsies showed a perifollicular CD4-positive T-lymphocytic infiltrate with pilotropism, intrafollicular mucin deposition, foreign-body granulomatous inflammation, acute inflammation, and follicular epithelial necrosis. None had concurrent systemic mycosis fungoides.
LIMITATIONS
Small retrospective cohort study.
CONCLUSION
We present these cases to expand the clinical and histopathologic spectrum of FMF that may strikingly resemble acneiform disorders and to highlight the importance of diagnostic reconsideration with histopathologic evaluation.
Topics: Acne Vulgaris; Adult; Aged; Biomarkers, Tumor; Biopsy; Cross-Sectional Studies; Diagnosis, Differential; Female; Hair Follicle; Humans; Immunohistochemistry; Male; Middle Aged; Mycosis Fungoides; Neoplasm Staging; Predictive Value of Tests; Retrospective Studies; Skin Neoplasms
PubMed: 33492839
DOI: 10.1097/DAD.0000000000001698 -
The American Journal of Dermatopathology Sep 2015Mycosis fungoides (MF), the most common form of cutaneous lymphoma is derived from postthymic T cells that migrate to the skin likely under the influence of chronic...
Mycosis fungoides (MF), the most common form of cutaneous lymphoma is derived from postthymic T cells that migrate to the skin likely under the influence of chronic antigen stimulation. Less common histomorphologic variants are diagnostically challenging because of their resemblance to reactive conditions. Three men aged 46, 73, and 74 years and one 83-year-old woman were encountered in the files of one of the authors and represented the patients. The patients had a longstanding eruption for several years. In 2 cases, the clinical course was aggressive with extracutaneous lymph node and/or peripheral blood dissemination. One patient had vesicles noted clinically. In all cases, vesiculation was a prominent feature histologically, which lead to an erroneous categorization initially as a reactive process. Basilar colonization by cerebriform lymphocytes along with the mucinous quality of the vesicle was diagnostic clue histologically, whereas the phenotypic and molecular profile was typical for MF. Strong expression of interleukin 5 and interleukin 10 in atypical lymphocytes in comparison with interferon gamma suggested a T-helper type 2-dominant cytokine microenvironment. In reviewing the literature of the 6 previously reported cases, 3 patients died of the disease; all these patients had vesicular lesions both clinically and histologically. We concluded that vesicular MF is a distinct histological and in some instances clinical variant of MF. The correlation of the vesicular eczematous quality of the eruption and a more aggressive clinical course may reflect the skewing toward a T-helper type 2-dominant cytokine milieu typical of advanced disease.
Topics: Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Mycosis Fungoides; Skin Neoplasms
PubMed: 25545185
DOI: 10.1097/DAD.0000000000000242 -
Best Practice & Research. Clinical... Sep 2019Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common types of cutaneous lymphoma, accounting for approximately 60% of cutaneous T-cell lymphomas.... (Review)
Review
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common types of cutaneous lymphoma, accounting for approximately 60% of cutaneous T-cell lymphomas. Diagnosis requires correlation of clinical, histologic, and molecular features. A multitude of factors have been linked to the aetiopathogenesis, however, none have been definitively proven. Erythrodermic MF (E-MF) and SS share overlapping clinical features, such as erythroderma, but are differentiated on the degree of malignant blood involvement. While related, they are considered to be two distinct entities originating from different memory T cell subsets. Differential expression of PD-1 and KIR3DL2 may represent a tool for distinguishing MF and SS, as well as a means of monitoring treatment response. Treatment of E-MF/SS is guided by disease burden, patients' ages and comorbidities, and effect on quality of life. Current treatment options include biologic, targeted, immunologic, and investigational therapies that can provide long term response with minimal side effects. Currently, allogeneic stem cell transplantation is the only potential curative treatment.
Topics: Humans; Immunologic Memory; Mycosis Fungoides; Neoplasm Proteins; Programmed Cell Death 1 Receptor; Receptors, KIR3DL2; Sezary Syndrome; Skin Neoplasms; T-Lymphocytes
PubMed: 31585624
DOI: 10.1016/j.beha.2019.06.004 -
Current Oncology Reports Mar 2018Cutaneous T-cell lymphoma (CTCL) is a rare form of non-Hodgkin lymphoma. Globally, the most common subtypes of CTCL are mycosis fungoides and Sézary syndrome. CTCL can... (Review)
Review
PURPOSE OF REVIEW
Cutaneous T-cell lymphoma (CTCL) is a rare form of non-Hodgkin lymphoma. Globally, the most common subtypes of CTCL are mycosis fungoides and Sézary syndrome. CTCL can confer significant morbidity and even mortality in advanced disease. Here we review the current and potential future treatments for advanced-stage CTCL.
RECENT FINDINGS
Heterogeneity of treatment choice has been demonstrated both in US and non-US centers. Systemic treatment choice is currently guided by prognostic features, incorporating stage, immunophenotypic and molecular findings, and patient-specific factors such as age and comorbidities. Randomized controlled studies are uncommon, and the literature is composed predominantly of retrospective, cohort, and early-phase studies. International consensus guidelines are available; however, the lack of comparative trials means that there is no clear algorithmic approach to treatment. This review article reports on the systemic treatment options in current use for advanced CTCL, and on the possible future therapies, acknowledging that an algorithmic approach is not yet forthcoming to guide treatment prioritization.
Topics: Combined Modality Therapy; Humans; Mycosis Fungoides; Prognosis; Sezary Syndrome; Skin Neoplasms
PubMed: 29572582
DOI: 10.1007/s11912-018-0678-x -
Cutis Jun 2015In recent years, the distinction between idiopathic follicular mucinosis (FM) and lymphoma-associated follicular mucinosis (LAFM) has been made through assessment of... (Review)
Review
In recent years, the distinction between idiopathic follicular mucinosis (FM) and lymphoma-associated follicular mucinosis (LAFM) has been made through assessment of T-cell receptor gene rearrangement, flow cytometry, and immunohistochemistry. These methods, among others, have mostly identified monoclonality as a defining characteristic of LAFM; however, this finding cannot be considered conclusive, as monoclonality also has been described in benign inflammatory dermatoses such as lichen planus and idiopathic FM. Pure histologic diagnosis also is unreliable in many cases, as the histologic patterns of idiopathic FM and LAFM overlap. In this article, we discuss the importance of close clinical follow-up in patients with patch-stage mycosis fungoides (MF) or FM who have had a nondiagnostic histopathologic evaluation. We also highlight the value of ancillary testing, including T-cell receptor gene rearrangement, flow cytometry, and immunohistochemistry, as a component in the diagnostic process rather than the sole diagnostic moiety. Diagnosis and classification of idiopathic FM and LAFM continue to pose challenges for dermatologists, oncologists, and pathologists, and no single diagnostic tool is sufficient in providing diagnostic certainty; rather, a collective evaluation of pathologic, molecular, and clinical criteria is required. Currently, classification of idiopathic FM and LAFM incorporates clinical information and histologic assessment, but little consideration is given to the implications of the diagnosis from the patient's perspective. Revisiting histologic classification of these entities while incorporating the patient's perspective may prove beneficial to dermatologists as well as patients.
Topics: Diagnosis, Differential; Flow Cytometry; Gene Rearrangement; Humans; Immunohistochemistry; Mucinosis, Follicular; Mycosis Fungoides; Receptors, Antigen, T-Cell; Skin Neoplasms
PubMed: 26125224
DOI: No ID Found