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JAMA Dermatology Oct 2023There are limited prognostic statistics and data available on survival outcomes for patients with mycosis fungoides (MF) in Asia.
IMPORTANCE
There are limited prognostic statistics and data available on survival outcomes for patients with mycosis fungoides (MF) in Asia.
OBJECTIVE
To determine the prognostic factors and survival outcomes of patients with MF among a cohort in China.
DESIGN, SETTING, AND PARTICIPANTS
This was a retrospective cohort study of patients with MF who received treatment at a tertiary referral center for skin lymphoma (Peking University First Hospital, Beijing, China) from August 1, 2009, to August 31, 2021. Data were analyzed from September 1, 2021, to December 31, 2022.
MAIN OUTCOMES AND MEASURES
Overall survival (OS), disease-specific survival (DSS), and progression-free survival (PFS); for prognostic factors, hazard ratios (HRs), and adjusted HRs (aHRs; adjusted for sex, age, and overall TNMB [tumor, node, metastasis, blood] stage) determined using the Cox proportional hazards model.
RESULTS
The study cohort comprised 461 patients with MF (median [range] age at diagnosis, 46 [5-87] years; 275 [59.7%] men and 186 [40.3%] women; 461 [100%] Chinese). The overall 5-year rate was 82.2% for OS, 83.5% for DSS, and 79.6% for PFS. Stage-specific 5-year OS rates were 95.7% for stage IA, 93.2% for IB, 95.7% for IIA, 70.1% for IIB, 55.3% for III, and 23.6% for IV. Compared with a UK cohort, our Chinese cohort had a younger median age at diagnosis (46 years vs 54 years) and a more favorable 5-year OS (82.2% vs 75.0%); however, after adjusting for age, the discrepancy in the 5-year OS rate was diminished (77.3% vs 76.4%). Cox models revealed that unfavorable predictors of OS, PFS, and DSS, respectively, were: age older than 60 years (aHR [95% CI], 2.25 [1.28-3.96]; 2.09 [1.16-3.76]; 2.27 [1.39-3.72]); advanced TNMB stage; advanced overall stage; large-cell transformation (aHR [95% CI], 2.16 [1.17-3.99]; 2.29 [1.21-4.33]; 2.21 [1.26-3.86]); and elevated lactate dehydrogenase levels (aHR [95% CI], 3.92 [1.64-9.36]; 4.77 [1.86-12.22]; 5.05 [2.23-11.42]). Biological sex and plaque lesion type were not associated with prognosis among this study cohort.
CONCLUSION AND RELEVANCE
The findings of this retrospective cohort study of patients with MF in China suggest that Asian patients are diagnosed at a younger age and have a higher 5-year OS compared with patients of other races in studies in other countries (predominantly White). Prognostic factors were similar to those of previous studies, except for patient sex and plaque lesion type.
Topics: Male; Humans; Female; Child, Preschool; Child; Adolescent; Young Adult; Adult; Middle Aged; Aged; Aged, 80 and over; Prognosis; Sezary Syndrome; Retrospective Studies; Neoplasm Staging; Disease Progression; Mycosis Fungoides; Skin Neoplasms; China
PubMed: 37585188
DOI: 10.1001/jamadermatol.2023.2634 -
Annals of Diagnostic Pathology Aug 2020Mycosis Fungoides (MF) is known as 'the great mimicker' due to its capacity to emulate several dermatoses, both in the clinic and on histology. This often leads to the... (Review)
Review
Mycosis Fungoides (MF) is known as 'the great mimicker' due to its capacity to emulate several dermatoses, both in the clinic and on histology. This often leads to the diagnosis being missed or delayed, which consequently leads to poorer prognosis. For a timely diagnosis, it is crucial that the physician is aware of the various clinical and histological presentations of MF, as well as the proper diagnostic protocols. In the current review, we concisely encapsulate all the variants of MF as well has the conditions it mimics clinically and histologically. Through this, we aim to provide clinicians with a holistic picture of MF and help them determine when to suspect this disease and steps to take in order to nail the diagnosis.
Topics: Abscess; Biopsy; Dermatology; Diagnosis, Differential; Female; High Mobility Group Proteins; Humans; Lymphocytes; Lymphoma, T-Cell, Cutaneous; Male; Middle Aged; Mycosis Fungoides; Neoplasm Staging; Pathology; Practice Guidelines as Topic; Skin Diseases; Skin Neoplasms
PubMed: 32554312
DOI: 10.1016/j.anndiagpath.2020.151546 -
Blood Jan 2022Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, undergo large-cell transformation (LCT) in the late stage, manifesting aggressive behavior,...
Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, undergo large-cell transformation (LCT) in the late stage, manifesting aggressive behavior, resistance to treatments, and poor prognosis, but the mechanisms involved remain unclear. To identify the molecular driver of LCT, we collected tumor samples from 133 MF patients and performed whole-transcriptome sequencing on 49 advanced-stage MF patients, followed by integrated copy number inference and genomic hybridization. Tumors with LCT showed unique transcriptional programs and enriched expressions of genes at chr7q. Paternally expressed gene 10 (PEG10), an imprinted gene at 7q21.3, was ectopically expressed in malignant T cells from LCT, driven by 7q21.3 amplification. Mechanistically, aberrant PEG10 expression increased cell size, promoted cell proliferation, and conferred treatment resistance by a PEG10/KLF2/NF-κB axis in in vitro and in vivo models. Pharmacologically targeting PEG10 reversed the phenotypes of proliferation and treatment resistance in LCT. Our findings reveal new molecular mechanisms underlying LCT and suggest that PEG10 inhibition may serve as a promising therapeutic approach in late-stage aggressive T-cell lymphoma.
Topics: Animals; Apoptosis Regulatory Proteins; Cell Line, Tumor; Cell Transformation, Neoplastic; DNA-Binding Proteins; Female; Gene Amplification; Gene Expression Regulation, Neoplastic; Genomic Imprinting; Humans; Lymphoma, T-Cell, Cutaneous; Mice, Inbred NOD; Mice, SCID; Mycosis Fungoides; RNA-Binding Proteins; Skin Neoplasms; Mice
PubMed: 34582557
DOI: 10.1182/blood.2021012091 -
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 -
Pediatric Dermatology Jan 2020Mycosis fungoides (MF) is the most common primary cutaneous lymphoma in pediatric patients. Given the indolent nature of MF, symptoms often present in childhood but may... (Review)
Review
Mycosis fungoides (MF) is the most common primary cutaneous lymphoma in pediatric patients. Given the indolent nature of MF, symptoms often present in childhood but may not be diagnosed as MF until adulthood. Delayed diagnosis is associated with poor long-term prognosis. Thus, increased clinician recognition and accurate diagnosis of early-stage MF in pediatric patients is critically important. In this review, we summarize the clinical features of the most common pediatric MF subtypes and highlight important differences between pediatric and adult MF. Moreover, we reviewed all pediatric MF case series published between 2008 and 2018 to analyze treatment modalities and identify emerging therapies. As treatment of pediatric MF is complex, selection of therapy varies significantly depending upon the specific clinical characteristics, disease severity, and patients' preferences.
Topics: Biopsy; Child; Diagnosis, Differential; Humans; Mycosis Fungoides; Neoplasm Staging; Prognosis; Skin Neoplasms
PubMed: 31630432
DOI: 10.1111/pde.14026 -
Cells Jan 2022Cutaneous T-Cell Lymphomas (CTCL) presents with substantial clinical variability and transcriptional heterogeneity. In the recent years, several studies paved the way to... (Review)
Review
Cutaneous T-Cell Lymphomas (CTCL) presents with substantial clinical variability and transcriptional heterogeneity. In the recent years, several studies paved the way to elucidate aetiology and pathogenesis of CTCL using sequencing methods. Several T-cell subtypes were suggested as the source of disease thereby explaining clinical and transcriptional heterogeneity of CTCL entities. Several differentially expressed pathways could explain disease progression. However, exogenous triggers in the skin microenvironment also seem to affect CTCL status. Especially was shown to contribute to disease progression. Only little is known about the complex microbiome patterns involved in CTCL and how microbial shifts might impact this malignancy. Nevertheless, first hints indicate that the microbiome might at least in part explain transcriptional heterogeneity and that microbial approaches could serve in diagnosis and prognosis. Shaping the microbiome could be a treatment option to maintain stable disease. Here, we review current knowledge of transcriptional heterogeneity of and microbial influences on CTCL. We discuss potential benefits of microbial applications and microbial directed therapies to aid patients with CTCL burden.
Topics: Disease Progression; Humans; Lymphoma, T-Cell, Cutaneous; Microbiota; Mycosis Fungoides; Sezary Syndrome; Skin Neoplasms; Tumor Microenvironment
PubMed: 35159138
DOI: 10.3390/cells11030328 -
Indian Journal of Dermatology,...Background Follicular mycosis fungoides is a distinct variant of mycosis fungoides with a broad clinical spectrum. Recently, many studies have indicated that follicular...
Background Follicular mycosis fungoides is a distinct variant of mycosis fungoides with a broad clinical spectrum. Recently, many studies have indicated that follicular mycosis fungoides should be divided into different subtypes with disparate prognoses. Objective To define the clinicohistopathologic features and outcomes of follicular mycosis fungoides and to identify risk factors that may be related to the prognosis of Chinese patients with follicular mycosis fungoides. Materials and methods We conducted a single-centre retrospective study and reviewed the clinical, histopathologic and immunophenotypic data of 12 patients diagnosed with follicular mycosis fungoides between 2009 and 2020 in the Department of Dermatology of West China Hospital of Sichuan university. Results A total of 12 patients (seven males and five females) with a mean age of 30 ± 14 years (age range 16-55 years) were included. Scalp and face were the most common involved sites (100%). Follicular papules, acneiform lesions, plaques, and nodules, were the main clinical presentations. Histopathological findings were consistent with the classic manifestations of follicular mycosis fungoides, including folliculotropism, perifollicular and intrafollicular lymphocytic infiltrates and mucinous degeneration. Interferon α-1b was the most common treatment. Four patients died of follicular mycosis fungoides in three years. Notably, immunohistochemical analysis revealed a decreased number of CD20+ cells in the deceased patients. Limitations This is a retrospective evaluation with a small number of cases; further prospective studies are warranted to support our inferences. Conclusion Our patients were much younger than in previous studies. The observed difference in this cohort may be explained by race, in addition to the limited number of cases. A decreased number of B cells might be associated with a poor prognosis, and more studies are necessary to discover the role of B cells in follicular mycosis fungoides as well as in mycosis fungoides.
Topics: Male; Female; Humans; Adolescent; Young Adult; Adult; Middle Aged; Retrospective Studies; Skin Neoplasms; Mycosis Fungoides; Prognosis; China
PubMed: 37317772
DOI: 10.25259/IJDVL_1003_2021 -
Experimental Dermatology Feb 2023Mycosis fungoides (MF) is characterised by malignant CD4 T-cell infiltrates in the skin. The functional characteristics of the malignant T cells and their interaction...
Mycosis fungoides (MF) is characterised by malignant CD4 T-cell infiltrates in the skin. The functional characteristics of the malignant T cells and their interaction with the tumor immune microenvironment is largely unknown. We performed tape stripping of the stratum corneum (SC), a non-invasive technique, to gain insight into the cytokine secretion patterns in MF skin lesions. In addition, we assessed whether the SC cytokine profile of MF lesions is distinct from that of atopic dermatitis (AD) lesions. We compared nine cytokine levels in 20 patients with MF, 10 patients with AD and 10 healthy controls. In patients with MF and AD, lesional SC levels of IL-8 and MMP9 were significantly higher than in non-lesional SC and in healthy controls. VEGFα was significantly higher in lesional MF and AD skin than in healthy controls. The SC levels of IL-1α were significantly lower in MF and AD lesions than in healthy controls. There was no specific cytokine profile or inflammation pattern that could reliably distinguish MF from AD. In conclusion, in lesional SC of MF patients, pro-inflammatory cytokines can be detected. As a diagnostic method, tape stripping of lesional SC cannot discriminate MF skin from AD skin.
Topics: Humans; Mycosis Fungoides; Skin; Epidermis; Dermatitis, Atopic; Skin Neoplasms; Tumor Microenvironment
PubMed: 36302170
DOI: 10.1111/exd.14694 -
International Journal of Dermatology Dec 2022Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. However, it is rare in pediatric population. Most of the cases of pediatric MF present with... (Review)
Review
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. However, it is rare in pediatric population. Most of the cases of pediatric MF present with hypopigmented patches and/or various other forms, which may often mimic common childhood dermatoses, thereby causing a delay in the diagnosis. There are no established treatment guidelines for pediatric MF. As the progression of childhood MF is extremely rare and it has an indolent course, it is usually diagnosed at an early stage (IA, IB, IIA), and hence phototherapy with a response rate of >80% is a well-established effective treatment in children. However, as recurrences are frequently seen on stopping the therapies, a maintenance regimen and long-term follow-up is equally important. This article reviews the epidemiological factors, clinical presentations, diagnosis, and various treatment modalities used in pediatric MF. We analyzed and compared the data of almost 616 childhood MF cases from various studies undertaken from 1988 to 2021.
Topics: Child; Humans; Skin Neoplasms; Mycosis Fungoides; Lymphoma, T-Cell, Cutaneous; Hypopigmentation; Phototherapy
PubMed: 35193164
DOI: 10.1111/ijd.16098 -
Actas Dermo-sifiliograficas Mar 2020Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma in adults and children. The prevalence has increased in some countries, but no descriptive...
BACKGROUND
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma in adults and children. The prevalence has increased in some countries, but no descriptive studies of MF in the pediatric population have been done in Colombia to date.
METHODS
A combined prospective-retrospective study of 128 patients with a diagnosis of MF confirmed by the dermatology department and dermatopathology laboratory of Universidad de Antioquia between 2008 and 2017. We describe the clinical and histopathologic variants, response to treatment, and progression of the disease in 23 patients under 18 years of age.
RESULTS
The pediatric cases of MF accounted for 18% of all the cases on record. The median age of onset of lesions was 9 years, the median age at diagnosis was 11 years, and the median time between onset of lesions and diagnosis was 2 years. All patients were in early stages of the disease. Hypopigmented MF was the most common clinical presentation (in 52.2%), followed by classical MF (in 30.4%). Folliculotropic MF was identified in 17.4%. All patients were treated with topical corticosteroids and phototherapy. One patient received chemotherapy while still in the early stage of disease. Complete remission was achieved in 59.1% and a partial response in 40.9%. Only 2 patients remained asymptomatic for 5 years.
CONCLUSION
We found hypopigmented MF to be the most common clinical presentation in patients under 18 years of age. The disease did not progress to advanced stages in any of the patients, although recurrence after treatment interruption was common.
Topics: Administration, Topical; Adolescent; Adrenal Cortex Hormones; Age of Onset; Child; Child, Preschool; Colombia; Disease Progression; Female; Humans; Hypopigmentation; Male; Mycosis Fungoides; Phototherapy; Prospective Studies; Recurrence; Remission Induction; Retrospective Studies
PubMed: 31277835
DOI: 10.1016/j.ad.2019.04.004