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Acta Dermato-venereologica Jul 2023Mycosis fungoides is a rare cutaneous lymphoma in the paediatric population. The aim of this study was to examine the epidemiological, clinical, and histological...
Mycosis fungoides is a rare cutaneous lymphoma in the paediatric population. The aim of this study was to examine the epidemiological, clinical, and histological characteristics, as well as the treatment modalities and response to therapy of paediatric patients with mycosis fungoides. This retrospective cohort study reviewed the records of 37 paediatric patients treated at Rambam Medical Center, Israel, between 2013 and 2021. Extracted data included epidemiology, clinical presentation, histological reports, infiltrate clonality status, treatment modalities and response to therapy. The mean follow-up period was 60 months. All patients were diagnosed with stage IA or IB disease. Folliculotropic mycosis fungoides was the most prevalent variant (49%). Most patients were treated with phototherapy (90%), with a response rate of 85%, and a complete response rate of 55% after the first course. There were no significant differences in response to phototherapy between the folliculotropic or other variants (p = 0.072). Similarly, delayed diagnosis, atopic diathesis, clonality, phototherapy type or number of treatments, were not associated with response to therapy, while protracted phototherapy was associated with prolonged remission. In conclusion, mycosis fungoides in the paediatric population is an indolent disease with a favourable prognosis and potentially prolonged response to phototherapy.
Topics: Humans; Child; Retrospective Studies; Treatment Outcome; Mycosis Fungoides; Skin Neoplasms; Lymphoma, T-Cell, Cutaneous
PubMed: 37449370
DOI: 10.2340/actadv.v103.6557 -
Current Treatment Options in Oncology Sep 2021Choice of therapy in mycosis fungoides is based on both patient- and lymphoma-specific factors, such as disease characteristics, comorbidities, symptoms and effect on... (Review)
Review
Choice of therapy in mycosis fungoides is based on both patient- and lymphoma-specific factors, such as disease characteristics, comorbidities, symptoms and effect on quality of life, potential associated toxicities of therapy, response and tolerance to prior lines of therapy, and convenience and practicality. Generally, we sequence therapies from least toxic, targeted, nonimmunosuppressive to more toxic, immunosuppressive and from single agent to multiple agents, as necessary. If more toxic, immunosuppressive agents are required to alleviate disease burden or symptoms, we generally use them just long enough to control the disease, then transition to a maintenance regimen with less toxic, less immunosuppressive agents.
Topics: Biomarkers, Tumor; Clinical Decision-Making; Combined Modality Therapy; Disease Management; Disease Susceptibility; Humans; Mycosis Fungoides; Neoplasm Grading; Neoplasm Staging; Skin Neoplasms; Treatment Outcome
PubMed: 34570278
DOI: 10.1007/s11864-021-00899-0 -
Advances in Anatomic Pathology Nov 2015Mycosis fungoides (MF) is the most common type of cutaneous lymphoma, accounting for almost 50% of all primary cutaneous lymphomas. When initially described, it was... (Review)
Review
Mycosis fungoides (MF) is the most common type of cutaneous lymphoma, accounting for almost 50% of all primary cutaneous lymphomas. When initially described, it was believed to be a distinct clinical entity with a pathognomonic histopathologic picture. Through the years we have come to know that, like syphilis, MF is a great masquerader and can present clinically and histopathologically in many ways. This review is an attempt to cover the many faces of MF that have evolved through the years.
Topics: History, 19th Century; History, 20th Century; History, 21st Century; Humans; Mycosis Fungoides; Skin Neoplasms
PubMed: 26452212
DOI: 10.1097/PAP.0000000000000092 -
Clinical Journal of Oncology Nursing Oct 2021Mycosis fungoides and Sézary syndrome are the most common non-Hodgkin lymphomas that manifest primarily in the skin. Although early-stage disease has an excellent... (Review)
Review
BACKGROUND
Mycosis fungoides and Sézary syndrome are the most common non-Hodgkin lymphomas that manifest primarily in the skin. Although early-stage disease has an excellent long-term survival rate, advanced disease carries a poor survival rate. Given the lengthy and complex clinical course, nurses are at the forefront of education and supportive care management for patients and caregivers.
OBJECTIVES
This article aims to provide an overview of mycosis fungoides and Sézary syndrome and to highlight practice considerations for optimal nursing care.
METHODS
Clinical presentation, diagnosis, management, and nursing consideration are discussed.
FINDINGS
Oncology nurses have a vital role in educating patients and their caregivers about the side effects of cancer treatment, appropriate skin care, and infection risk.
Topics: Humans; Lymphoma, Non-Hodgkin; Mycosis Fungoides; Oncology Nursing; Sezary Syndrome; Skin Neoplasms
PubMed: 34533520
DOI: 10.1188/21.CJON.555-562 -
The Australasian Journal of Dermatology Feb 2021Primary cutaneous lymphomas represent a heterogeneous group of T- and B-cell lymphomas with distinct clinical presentations, histopathologic features, treatment... (Review)
Review
Primary cutaneous lymphomas represent a heterogeneous group of T- and B-cell lymphomas with distinct clinical presentations, histopathologic features, treatment approaches and outcomes. The cutaneous T-cell lymphomas, which include mycosis fungoides and Sézary syndrome, account for the majority of the cutaneous lymphomas. This Clinical Practice Statement is reflective of the current clinical practice in Australia. An expanded form of the Clinical Practice Statement (and updates), along with helpful patient resources and access to support groups, can be found at the following (http://www.australasianlymphomaalliance.org.au).
Topics: Biopsy; Hematologic Tests; Humans; Mycosis Fungoides; Neoplasm Staging; Prognosis; Sezary Syndrome; Skin; Skin Neoplasms; Survival Rate
PubMed: 33368169
DOI: 10.1111/ajd.13467 -
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 -
Seminars in Cutaneous Medicine and... Mar 2018Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, which typically presents with erythematous patches and plaques, histopathologically... (Review)
Review
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, which typically presents with erythematous patches and plaques, histopathologically characterized by superficial infiltrates of small to mediumsized atypical epidermotropic T cells. Apart from this classic type of MF, many clinical and/or histopathologic variants have been described. Correct diagnosis of these MF variants is important, but may be difficult, because they may mimic a wide variety of inflammatory skin diseases. In this review, clinical and histopathologic characteristics of distinct variants of MF are presented, and their differential diagnosis and therapeutic options are discussed.
Topics: Diagnosis, Differential; Humans; Mycosis Fungoides; Skin Neoplasms
PubMed: 29719015
DOI: 10.12788/j.sder.2018.004 -
Anais Brasileiros de Dermatologia 2021Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis... (Review)
Review
Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.
Topics: Humans; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Quality of Life; Sezary Syndrome; Skin Neoplasms
PubMed: 34053802
DOI: 10.1016/j.abd.2020.12.007 -
Current Hematologic Malignancy Reports Jun 2017Diagnosis and management of mycosis fungoides and Sézary syndrome (MF/SS) require accurate clinicopathological correlation and a multidisciplinary approach. We reviewed... (Review)
Review
PURPOSE OF REVIEW
Diagnosis and management of mycosis fungoides and Sézary syndrome (MF/SS) require accurate clinicopathological correlation and a multidisciplinary approach. We reviewed major advances in the field regarding diagnostic and prognostic tools as well as skin-directed therapies (SDTs) and systemic agents for MF/SS published in the past 2 years.
RECENT FINDINGS
Improved technology (T-cell receptor high-throughput sequencing) and increased multicenter collaboration (Cutaneous Lymphoma International Consortium) have led to diagnostic/prognostic advances. Concurrently, numerous genomic studies have enhanced understanding of disease pathogenesis. Advances in SDTs include topical resiquimod, a novel potent Toll-like receptor (TLR) agonist; consensus CTCL phototherapy guidelines; and use of low-dose radiation therapy. Novel systemic therapies for advanced disease of note include targeted antibody drug conjugates (brentuximab vedotin), immune checkpoint inhibitors, and allogeneic hematopoietic stem cell transplantation (HSCT). Our "toolbox" to diagnose and treat the spectrum of MF/SS continues to expand. Further characterization of genomic data going forward will enable a rational approach to selecting and combining therapies to improve patient care.
Topics: Biomarkers, Tumor; Combined Modality Therapy; Disease Management; Genomics; Humans; Mycosis Fungoides; Prognosis; Sezary Syndrome; Skin Neoplasms; Treatment Outcome
PubMed: 28540671
DOI: 10.1007/s11899-017-0387-9 -
Experimental Dermatology Oct 2018Primary cutaneous T-cell lymphoma (CTCL) comprises the second most common group of extra-nodal non-Hodgkin's lymphoma. They represent incurable primary extra-nodal... (Review)
Review
Primary cutaneous T-cell lymphoma (CTCL) comprises the second most common group of extra-nodal non-Hodgkin's lymphoma. They represent incurable primary extra-nodal lymphomas of major T cells, uniformly present in the skin with 1%-2% risk of systemic dissemination in mycosis fungoides (MF), which represents the most common subtype of CTCL. In general, long-term antigen stimulation is thought, through key cytokine signalling pathways, to induce an inflammatory response with T-cell proliferation, leading to a clonal malignant T cell with continuous expansion. However, in recent years, using data harvested from high-throughput transcriptional profiling, substantial advances in the understanding of the molecular pathogenesis were made to understand the complex pathogenesis of CTCL. In this review, the actual data are summarised.
Topics: Cell Proliferation; Humans; MicroRNAs; Mycosis Fungoides; Sezary Syndrome; Signal Transduction; Skin Neoplasms; T-Lymphocyte Subsets
PubMed: 29862563
DOI: 10.1111/exd.13701