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Cutis May 2021
Topics: Black or African American; Humans; Mycosis Fungoides; Skin Neoplasms
PubMed: 34288846
DOI: 10.12788/cutis.0247 -
Dermatology (Basel, Switzerland) 2021Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphoma (CTCL). There is currently no cure for CTCL, and treatment is...
BACKGROUND
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphoma (CTCL). There is currently no cure for CTCL, and treatment is aimed at limiting disease progression. This study evaluated the efficacy and tolerability of alitretinoin in CTCL management.
METHODS
A retrospective, multicenter study was conducted on CTCL patients treated with alitretinoin as a primary agent or in combination with standard therapies.
RESULTS
Forty-eight patients with MF (n = 40) and SS (n = 8) with a median age of 59.7 years (±14.3) were eligible for study inclusion. Treatment response data were evaluated in 40 patients and safety in 42 patients. 40.0% of the patients had early-stage, 43.8% had advanced-stage CTCL, and in 16.7% of patients there was insufficient information for staging. 40.0% (16/40) of the patients achieved a complete or partial response, whereas 47.5% (19/40) achieved stable disease, 12.5% (5/40) had progressive disease, and there were no cases of disease relapses in responders. Both early and advanced stages of CTCL were responsive to alitretinoin as a primary or combined modality. Alitretinoin was well tolerated, and 64.3% (27/42) of patients did not report any side effects. The most commonly observed side effect was hypertriglyceridemia.
CONCLUSIONS
This retrospective analysis supports the efficacy and safety of alitretinoin in clearing skin disease and preventing disease progression in CTCL as a monotherapy or in combination with standard therapies.
Topics: Adult; Aged; Aged, 80 and over; Alitretinoin; Antineoplastic Agents; Canada; Combined Modality Therapy; Female; Humans; Male; Middle Aged; Mycosis Fungoides; Retrospective Studies; Sezary Syndrome; Skin Neoplasms; Treatment Outcome; Young Adult
PubMed: 33429396
DOI: 10.1159/000512484 -
Expert Review of Anticancer Therapy Jun 2020Advanced mycosis fungoides (MF) and Sézary syndrome (SS) are forms of cutaneous T-cell lymphoma characterized by a poor prognosis. Treatments are associated with high... (Comparative Study)
Comparative Study Review
INTRODUCTION
Advanced mycosis fungoides (MF) and Sézary syndrome (SS) are forms of cutaneous T-cell lymphoma characterized by a poor prognosis. Treatments are associated with high rates of relapse, and thus there exists an unmet medical need for new, improved therapies. Mogamulizumab is a novel defucosylated monoclonal antibody targeting C-C chemokine receptor 4 that eradicates malignant cells via antibody-dependent cellular cytotoxicity (ADCC).
AREAS COVERED
Phase I-III clinical trials involving mogamulizumab demonstrated its significant efficacy and tolerability. In the phase III MAVORIC study, mogamulizumab exhibited greater efficacy than vorinostat (response rate, 28% versus 5%; median progression-free survival, 7.7 versus 3.1 months, respectively) for advanced, relapsed/refractory MF/SS. Responses were durable (median, 14.1 months), and response rates were highest in SS (37%) and within the blood compartment (68%). Common adverse events include infusion reactions and drug eruptions. The drug also increases the risk of immune-mediated complications such as autoimmune disease and acute graft-versus-host disease following transplantation.
EXPERT OPINION
Mogamulizumab represents a valuable therapy for advanced MF/SS as it can produce prolonged responses, particularly within the peripheral blood. Since it eliminates malignant T cells via ADCC, combining mogamulizumab with immunotherapeutic agents such as interferons, interleukin-12, and Toll-like receptor agonists may further enhance its efficacy.
Topics: Antibodies, Monoclonal, Humanized; Antineoplastic Agents; Humans; Mycosis Fungoides; Prognosis; Progression-Free Survival; Sezary Syndrome; Skin Neoplasms
PubMed: 32320304
DOI: 10.1080/14737140.2020.1760096 -
Journal of the European Academy of... Jul 2021Dermoscopy and trichoscopy are non-invasive methods used as auxiliary tools in diagnostics of different dermatoses. To date, no systematic review concerning the utility... (Review)
Review
Dermoscopy and trichoscopy are non-invasive methods used as auxiliary tools in diagnostics of different dermatoses. To date, no systematic review concerning the utility of dermoscopy and trichoscopy in the diagnostics of primary cutaneous lymphomas has been published. The aim of this study was to summarize the current state of knowledge on this topic based on systematic search of PubMed database and related references published before 8th of August 2020. Besides dermoscopic features, type of dermoscope, polarization mode, magnification, number of cases and histopathological correlation were analysed. A total of 34 records were included into the final analysis, evaluating 141 patients diagnosed with primary cutaneous T-cell lymphomas and 70 patients with primary cutaneous B-cell lymphomas. Most of the analysed records evaluated dermoscopic features (n = 206); trichoscopy was analysed in only 5 cases. Structures most commonly observed in classical mycosis fungoides (n = 108) were fine short linear vessels/linear vessels, spermatozoa-like vessels and orange-yellow patchy areas. In folliculotropic mycosis fungoides (n = 12), most frequently observed were comedonal lesions/comedo openings/central keratotic plugs and white halo around hair follicles/perifollicular accentuation. Primary cutaneous marginal zone B-cell lymphoma (n = 42) and primary cutaneous follicle centre lymphoma (n = 20) most commonly presented with salmon-coloured background and fine short/linear irregular/serpentine vessels. For other PCL, with less than 10 cases reported in the analysed records, details have been provided in the article. Most observations analysed in this systematic review rely on findings from case reports/case series (with the level of evidence V) and lack a control group. A few studies provided information concerning technical aspects of dermoscopic/trichoscopic examination. The role of dermoscopy/trichoscopy in diagnostics of cutaneous lymphomas requires further studies, especially in entities where dermoscopic features have been described in only single or a few cases. However, it seems that this practical, accessory tool in future may provide additional clues during clinical assessment.
Topics: Dermoscopy; Humans; Lymphoma, B-Cell; Lymphoma, T-Cell, Cutaneous; Male; Mycosis Fungoides; Skin Neoplasms
PubMed: 33710688
DOI: 10.1111/jdv.17219 -
The American Journal of Dermatopathology Mar 2023Mycosis fungoides is rarely associated to B-cell malignancies, and the few reported cases are mainly internal lymphomas involving secondarily the skin (ie, chronic... (Review)
Review
BACKGROUND
Mycosis fungoides is rarely associated to B-cell malignancies, and the few reported cases are mainly internal lymphomas involving secondarily the skin (ie, chronic lymphocytic leukemia).
OBJECTIVES
The aim of our study is to describe the clinical and histopathological features of 4 patients presenting with 2 concurrent primary cutaneous lymphomas and review the pertinent literature.
METHODS
We identified 4 cases of concurrent primary cutaneous lymphomas in our institutions. An extracutaneous lymphoma was ruled out on the basis of a complete work out. We performed a PubMed search to identify reported cases of primary cutaneous composite or concurrent lymphomas.
RESULTS
Eleven cases of primary cutaneous concurrent lymphomas have been described in the literature. Counting all together (our cases and the cases previously described in the literature), mycosis fungoides was the most frequent primary cutaneous T-cell lymphoma (TCL) (13/15), followed by 1 case of peripheral TCL-NOS and 1 case of subcutaneous panniculitis-like TCL. Regarding the associated primary cutaneous B-cell lymphomas, 8/15 cases consisted of low-grade B-cell lymphomas [that is, 5 marginal zone lymphoma (in the most recent classification reclassified as marginal zone lymphoproliferative disorder, MZLD, 2 follicular-center B-cell lymphoma (primary cutaneous follicle-center lymphoma) and 1 low-grade NOS B-cell lymphoma]; 4/15 were associated to Epstein-Barr virus; 1 case consisted of a methotrexate-associated lymphoproliferative disease, and 2 cases consisted of primary cutaneous diffuse large B-cell lymphoma-leg type.
CONCLUSIONS
Primary cutaneous concurrent lymphomas are exceptional. Clinicopathological correlation and a complete workout to reach the correct diagnosis may guide the appropriate treatment in each case.
Topics: Humans; Epstein-Barr Virus Infections; Skin Neoplasms; Herpesvirus 4, Human; Mycosis Fungoides; Lymphoma, B-Cell, Marginal Zone; Lymphoma, T-Cell, Cutaneous
PubMed: 36728288
DOI: 10.1097/DAD.0000000000002378 -
Cells Mar 2024Cutaneous T-cell lymphoma (CTCL) is characterized by the proliferation of malignant T cells in inflamed skin lesions. Mycosis fungoides (MF)-the most common variant of... (Review)
Review
Cutaneous T-cell lymphoma (CTCL) is characterized by the proliferation of malignant T cells in inflamed skin lesions. Mycosis fungoides (MF)-the most common variant of CTCL-often presents with skin lesions around the abdomen and buttocks ("bathing suit" distribution), i.e., in skin areas devoid of sun-induced vitamin D. For decades, sunlight and vitamin D have been connected to CTCL. Thus, vitamin D induces apoptosis and inhibits the expression of cytokines in malignant T cells. Furthermore, CTCL patients often display vitamin D deficiency, whereas phototherapy induces vitamin D and has beneficial effects in CTCL, suggesting that light and vitamin D have beneficial/protective effects in CTCL. Inversely, vitamin D promotes T helper 2 (Th2) cell specific cytokine production, regulatory T cells, tolerogenic dendritic cells, as well as the expression of immune checkpoint molecules, all of which may have disease-promoting effects by stimulating malignant T-cell proliferation and inhibiting anticancer immunity. Studies on vitamin D treatment in CTCL patients showed conflicting results. Some studies found positive effects, others negative effects, while the largest study showed no apparent clinical effect. Taken together, vitamin D may have both pro- and anticancer effects in CTCL. The balance between the opposing effects of vitamin D in CTCL is likely influenced by treatment and may change during the disease course. Therefore, it remains to be discovered whether and how the effect of vitamin D can be tilted toward an anticancer response in CTCL.
Topics: Humans; Skin Neoplasms; Vitamin D; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Skin; Skin Diseases; Vitamins
PubMed: 38534347
DOI: 10.3390/cells13060503 -
Journal of the American Academy of... Jun 2022The clinicoprognostic implications of head and neck involvement of mycosis fungoides (MF) are poorly understood.
BACKGROUND
The clinicoprognostic implications of head and neck involvement of mycosis fungoides (MF) are poorly understood.
OBJECTIVES
To evaluate the association of head and neck involvement on the clinicoprognostic features of MF.
METHODS
The clinical features and survival outcomes of patients with MF in a Korean academic medical center database were retrospectively evaluated according to the presence of head and neck involvement at diagnosis.
FINDINGS
Cases of MF with (group A, n = 39) and without (group B, n = 85) head and neck involvement at diagnosis were identified. Advanced-stage disease (stages IIB-IVB) was more common in group A (43.6%) than in group B (5.9%) (P < .001). MF progression, extracutaneous dissemination, and large-cell transformation more commonly occurred in group A than in group B. The 10-year overall survival rate was worse in group A (53.4%) compared with group B (81.6%) (P < .001). Head and neck involvement at diagnosis was associated with poor prognosis in early-stage MF (stages IA-IIA) and was independently associated with worse progression-free survival (hazard ratio, 24.4; 95% confidence interval, 2.2-267.6; P = .009).
LIMITATIONS
A single center, retrospective design.
CONCLUSION
Head and neck involvement of MF was associated with a poor prognosis.
Topics: Cell Transformation, Neoplastic; Humans; Mycosis Fungoides; Neoplasm Staging; Prognosis; Retrospective Studies; Skin Neoplasms
PubMed: 33771590
DOI: 10.1016/j.jaad.2021.03.056 -
Presse Medicale (Paris, France : 1983) Mar 2022The therapeutic approach for mycosis fungoides, the most common type of primary cutaneous T-cell lymphoma, is based mainly on the stage of the disease, and skin-directed... (Review)
Review
The therapeutic approach for mycosis fungoides, the most common type of primary cutaneous T-cell lymphoma, is based mainly on the stage of the disease, and skin-directed treatment is recommended by all international guidelines as the first-line of treatment for early-stage disease. Skin-directed treatments may be also given in combination with systemic therapies in early-stage mycosis fungoides patients recalcitrant to different types of skin-directed treatments, or in certain patients with high-risk features. Advanced-stage mycosis fungoides is treated mainly with systemic treatments, which may be combined with skin-directed treatments. Due to the rarity of mycosis fungoides, controlled clinical trials of the different skin-directed treatment modalities are almost non-existent, with a few exceptions, and therefore recommendations are largely based on cohort studies and expert opinion. This paper reviews the new developments in skin-directed treatments and provides an update on new studies of already well-known therapies, and an update on novel treatments.
Topics: Administration, Cutaneous; Humans; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Skin Neoplasms
PubMed: 35562084
DOI: 10.1016/j.lpm.2022.104125 -
Anais Brasileiros de Dermatologia 2021Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. Most early-stage mycosis fungoides cases follow an indolent course, hence considered by doctors a...
BACKGROUND
Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. Most early-stage mycosis fungoides cases follow an indolent course, hence considered by doctors a relatively easy condition. However, since mycosis fungoides bears the title of cancer, patients might perceive it differently.
OBJECTIVE
To investigate patients' illness perception, and its relationships to quality of life, depression, anxiety, and coping among early-stage mycosis fungoides patients.
METHODS
A cross-sectional questionnaire-based study was conducted. Patients from a single tertiary medical center completed the Revised Illness Perception Questionnaire, the MF/SS-CTCL Quality of Life scale, the Hospital Anxiety and Depression Scale, and The Mental Adjustment to Cancer Scale.
RESULTS
Thirty patients (25 males, five females, mean age 51.60) with stage I mycosis fungoides were enrolled. Mycosis fungoides had a little impact on patients' daily life, quality of life, and levels of depression and anxiety, and they generally coped well. Disease understanding was low and was negatively correlated with impairment to quality of life and depression. Patients felt that stress and worry were features of the disease's etiology.
STUDY LIMITATIONS
A small sample of patients was included.
CONCLUSION
Patients with early-stage mycosis fungoides adapt well to their disease. Psychological interventions should be aimed at improving patients coping style and enhancing illness understanding, in order to maintain high quality of life.
Topics: Adaptation, Psychological; Cross-Sectional Studies; Female; Humans; Male; Middle Aged; Mycosis Fungoides; Perception; Quality of Life; Skin Neoplasms
PubMed: 33279315
DOI: 10.1016/j.abd.2020.05.008 -
The Cochrane Database of Systematic... Jul 2020Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, a malignant, chronic disease initially affecting the skin. Several therapies are available,... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, a malignant, chronic disease initially affecting the skin. Several therapies are available, which may induce clinical remission for a time. This is an update of a Cochrane Review first published in 2012: we wanted to assess new trials, some of which investigated new interventions.
OBJECTIVES
To assess the effects of interventions for MF in all stages of the disease.
SEARCH METHODS
We updated our searches of the following databases to May 2019: the Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, and LILACS. We searched 2 trials registries for additional references. For adverse event outcomes, we undertook separate searches in MEDLINE in April, July and November 2017.
SELECTION CRITERIA
Randomised controlled trials (RCTs) of local or systemic interventions for MF in adults with any stage of the disease compared with either another local or systemic intervention or with placebo.
DATA COLLECTION AND ANALYSIS
We used standard methodological procedures expected by Cochrane. The primary outcomes were improvement in health-related quality of life as defined by participants, and common adverse effects of the treatments. Key secondary outcomes were complete response (CR), defined as complete disappearance of all clinical evidence of disease, and objective response rate (ORR), defined as proportion of patients with a partial or complete response. We used GRADE to assess the certainty of evidence and considered comparisons of psoralen plus ultraviolet A (PUVA) light treatment as most important because this is first-line treatment for MF in most guidelines.
MAIN RESULTS
This review includes 20 RCTs (1369 participants) covering a wide range of interventions. The following were assessed as either treatments or comparators: imiquimod, peldesine, hypericin, mechlorethamine, nitrogen mustard and intralesional injections of interferon-α (IFN-α) (topical applications); PUVA, extracorporeal photopheresis (ECP: photochemotherapy), and visible light (light applications); acitretin, bexarotene, lenalidomide, methotrexate and vorinostat (oral agents); brentuximab vedotin; denileukin diftitox; mogamulizumab; chemotherapy with cyclophosphamide, doxorubicin, etoposide, and vincristine; a combination of chemotherapy with electron beam radiation; subcutaneous injection of IFN-α; and intramuscular injections of active transfer factor (parenteral systemics). Thirteen trials used an active comparator, five were placebo-controlled, and two compared an active operator to observation only. In 14 trials, participants had MF in clinical stages IA to IIB. All participants were treated in secondary and tertiary care settings, mainly in Europe, North America or Australia. Trials recruited both men and women, with more male participants overall. Trial duration varied from four weeks to 12 months, with one longer-term study lasting more than six years. We judged 16 trials as at high risk of bias in at least one domain, most commonly performance bias (blinding of participants and investigators), attrition bias and reporting bias. None of our key comparisons measured quality of life, and the two studies that did presented no usable data. Eighteen studies reported common adverse effects of the treatments. Adverse effects ranged from mild symptoms to lethal complications depending upon the treatment type. More aggressive treatments like systemic chemotherapy generally resulted in more severe adverse effects. In the included studies, CR rates ranged from 0% to 83% (median 31%), and ORR ranged from 0% to 88% (median 47%). Five trials assessed PUVA treatment, alone or combined, summarised below. There may be little to no difference between intralesional IFN-α and PUVA compared with PUVA alone for 24 to 52 weeks in CR (risk ratio (RR) 1.07, 95% confidence interval (CI) 0.87 to 1.31; 2 trials; 122 participants; low-certainty evidence). Common adverse events and ORR were not measured. One small cross-over trial found once-monthly ECP for six months may be less effective than twice-weekly PUVA for three months, reporting CR in two of eight participants and ORR in six of eight participants after PUVA, compared with no CR or ORR after ECP (very low-certainty evidence). Some participants reported mild nausea after PUVA but no numerical data were given. One participant in the ECP group withdrew due to hypotension. However, we are unsure of the results due to very low-certainty evidence. One trial comparing bexarotene plus PUVA versus PUVA alone for up to 16 weeks reported one case of photosensitivity in the bexarotene plus PUVA group compared to none in the PUVA-alone group (87 participants; low-certainty evidence). There may be little to no difference between bexarotene plus PUVA and PUVA alone in CR (RR 1.41, 95% CI 0.71 to 2.80) and ORR (RR 0.94, 95% CI 0.61 to 1.44) (93 participants; low-certainty evidence). One trial comparing subcutaneous IFN-α injections combined with either acitretin or PUVA for up to 48 weeks or until CR indicated there may be little to no difference in the common IFN-α adverse effect of flu-like symptoms (RR 1.32, 95% CI 0.92 to 1.88; 82 participants). There may be lower CR with IFN-α and acitretin compared with IFN-α and PUVA (RR 0.54, 95% CI 0.35 to 0.84; 82 participants) (both outcomes: low-certainty evidence). This trial did not measure ORR. One trial comparing PUVA maintenance treatment to no maintenance treatment, in participants who had already had CR, did report common adverse effects. However, the distribution was not evaluable. CR and OR were not assessable. The range of treatment options meant that rare adverse effects consequently occurred in a variety of organs.
AUTHORS' CONCLUSIONS
There is a lack of high-certainty evidence to support decision making in the treatment of MF. Because of substantial heterogeneity in design, missing data, small sample sizes, and low methodological quality, the comparative safety and efficacy of these interventions cannot be reliably established on the basis of the included RCTs. PUVA is commonly recommended as first-line treatment for MF, and we did not find evidence to challenge this recommendation. There was an absence of evidence to support the use of intralesional IFN-α or bexarotene in people receiving PUVA and an absence of evidence to support the use of acitretin or ECP for treating MF. Future trials should compare the safety and efficacy of treatments to PUVA, as the current standard of care, and should measure quality of life and common adverse effects.
Topics: Acitretin; Antineoplastic Agents; Bexarotene; Combined Modality Therapy; Humans; Immunologic Factors; Interferon-alpha; Mycosis Fungoides; Neoplasm Staging; PUVA Therapy; Photochemotherapy; Photopheresis; Randomized Controlled Trials as Topic; Skin Neoplasms
PubMed: 32632956
DOI: 10.1002/14651858.CD008946.pub3