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International Journal of Molecular... Mar 2024Extracorporeal photopheresis (ECP) is an apheresis procedure that is conventionally used as a first-line treatment for cutaneous and leukemic subtypes of T-cell... (Review)
Review
Extracorporeal photopheresis (ECP) is an apheresis procedure that is conventionally used as a first-line treatment for cutaneous and leukemic subtypes of T-cell lymphoma, such as Sezary's syndrome and mycosis fungoides. Over the past three decades, its immunotherapeutic properties have been tested on a variety of autoimmune conditions, including many dermatologic diseases. There is ample evidence of ECP's ability to modify leukocytes and alter cytokine production for certain dermatologic diseases that have been refractory to first-line treatments, such as atopic dermatitis. However, the evidence on the efficacy of ECP for the treatment of these dermatologic diseases is unclear and/or lacks sufficient evidence. The purpose of this paper is to review the literature on the utilization and clinical efficacy of ECP in the treatment of several [autoimmune] dermatologic diseases and discuss its applications, guidelines, recommendations, and future implementation for dermatologic diseases.
Topics: Humans; Photopheresis; Skin Neoplasms; Mycosis Fungoides; Blood Component Removal; Sezary Syndrome
PubMed: 38474257
DOI: 10.3390/ijms25053011 -
Journal of the American Academy of... Jun 2015Sézary syndrome is a cutaneous T-cell lymphoma characterized by erythroderma and leukemic involvement.
BACKGROUND
Sézary syndrome is a cutaneous T-cell lymphoma characterized by erythroderma and leukemic involvement.
OBJECTIVE
We sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma.
METHODS
Features of patients with Sézary syndrome and normal-appearing skin or stage-T1 patches, fulfilling Sézary syndrome hematologic criteria and with histologically documented disease in normal-appearing skin were collected. Expression of Sézary syndrome molecular biomarkers in peripheral blood and skin lymphocytes were studied.
RESULTS
Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with Sézary syndrome. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred.
LIMITATIONS
Retrospective design and small sample size are limitations.
CONCLUSION
Sézary syndrome without erythroderma is a rare entity that may have a better prognosis than classic Sézary syndrome.
Topics: Administration, Topical; Adrenal Cortex Hormones; Age Factors; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Biopsy, Needle; Combined Modality Therapy; Dermatitis, Exfoliative; Female; Follow-Up Studies; France; Humans; Immunohistochemistry; Male; Methotrexate; Middle Aged; Neoplasm Invasiveness; Neoplasm Staging; Photopheresis; Rare Diseases; Retrospective Studies; Risk Assessment; Sampling Studies; Sex Factors; Sezary Syndrome; Skin Neoplasms
PubMed: 25981000
DOI: 10.1016/j.jaad.2014.11.015 -
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 -
Hematological Oncology Jun 2023Cutaneous lymphomas are a heterogeneous group of several distinct entities of lymphoproliferative diseases. The diagnosis of a cutaneous lymphoma is a challenge, and it... (Review)
Review
Cutaneous lymphomas are a heterogeneous group of several distinct entities of lymphoproliferative diseases. The diagnosis of a cutaneous lymphoma is a challenge, and it is always the result of a careful analysis of several information's consisting of clinical history, clinical picture, histological and molecular analyses. For this reason, experts taking care of patients with a skin lymphoma need to know all the peculiar diagnostic elements very well, in order not to run into mistakes. In this article, we will focus the discussion on some issues as the skin biopsy (when and where). In addition, we will discuss the approach to the erythrodermic patient, whose differential diagnoses include mycosis fungoides, and Sézary syndrome, beside more frequent inflammatory conditions. Finally, we will address the issue of quality of life and the possible support of the suffering patient with a cutaneous lymphoma, well knowing that the current therapeutic possibilities are unfortunately limited.
Topics: Humans; Quality of Life; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Sezary Syndrome; Skin Neoplasms
PubMed: 37294972
DOI: 10.1002/hon.3147 -
Journal of Cutaneous Medicine and... 2019The onychodystrophies associated with Sézary syndrome (SzS) have rarely been described in the literature. We performed a retrospective analysis of SzS patients at a... (Review)
Review
BACKGROUND
The onychodystrophies associated with Sézary syndrome (SzS) have rarely been described in the literature. We performed a retrospective analysis of SzS patients at a single institution and compared our data with previous publications.
OBJECTIVES
The objectives of this study were to identify and describe the most frequent nail alterations in patients with SzS.
METHODS
A retrospective analysis was performed with some prospective observations at the University of Pittsburgh from 1989 to 2017.
RESULTS
We identified 54 patients with SzS out of 535 patients with cutaneous T-cell lymphoma. Nineteen patients with SzS had photos of their nail. All those patients exhibited some type of onychodystrophy. The most common types were paronychia (63.2%; 12/19), leukonychia (42.1%; 8/19), onycholysis (42.1%; 8/19), trachyonychia (31.6%; 6/19), and subungual hyperkeratosis (26.3; 5/19). Cluster analysis of our data in comparison with published data on the psoriatic nails indicated that while leukonychia, onycholysis, subungual hyperkeratosis, and nail discoloration were frequently observed in psoriasis, onychauxis, anonychia, distal notching, and onychoschizia occurred more commonly in patients with SzS.
CONCLUSIONS
The most common nail manifestations in SzS patients included paronychia, leukonychia, and onycholysis. The nail manifestations in SzS patients appeared to be heterogeneous, while onychauxis, anonychia, distal notching, and onychoschizia seem to be specific to SzS in comparison with psoriasis.
Topics: Aged; Female; Humans; Hypopigmentation; Keratosis; Male; Middle Aged; Nail Diseases; Nails, Malformed; Onycholysis; Paronychia; Prospective Studies; Psoriasis; Retrospective Studies; Sezary Syndrome; Skin Neoplasms
PubMed: 30917680
DOI: 10.1177/1203475419839937 -
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 -
Journal of the European Academy of... Aug 2019Limited information exists regarding survival of Asian patients with mycosis fungoides (MF) and Sézary syndrome (SS). (Review)
Review
BACKGROUND
Limited information exists regarding survival of Asian patients with mycosis fungoides (MF) and Sézary syndrome (SS).
OBJECTIVE
To evaluate the epidemiology, outcome and prognostic factors of these patients.
METHODS
A retrospective review of MF/SS cases diagnosed from 2000 to 2011 at a tertiary referral dermatology centre in Singapore was performed.
RESULTS
Of 246 patients, 63% were male and the median age at diagnosis was 49 years. 73.2% were Chinese, 12.6% Indian, 6.9% Malay and 7.3% Caucasian. A total of 239 patients (97.2%) had MF and seven had SS. Median follow-up duration was 6.3 years, and median duration of symptoms at diagnosis was 13 months. For patients with MF, the majority had early disease (92.8% stage IA-IIA). 3.8% were stage IIB, 1.7% stage III and 1.7% stage IV. Complete response to treatment occurred in 78.2%, partial response in 9.6%, persistent but non-progressive disease in 10.0% and disease progression in 4.1% of patients. Large cell transformation occurred in 4.1% of patients. Mean overall survival during this study was 12.7 years, with death occurring in 2.5% of patients (all ≥stage IIB at diagnosis). For patients with SS, 71.4% presented with stage IVA disease, 28.6% stage IVB. Complete response to treatment occurred in 14.2%, persistent but non-progressive disease in 28.6% and disease progression in 57.2% of patients. Mean overall survival was 3.3 years within this study, with death occurring in 42.9% of SS patients. Prognostic factors associated with favourable recurrence-free survival were male gender (P = 0.008), early disease stage (T1) at diagnosis (P < 0.001) and absence of maintenance treatment after remission (P = 0.01).
CONCLUSION
Compared to Caucasian and East Asian cohorts, MF in South-East Asians was diagnosed at a younger age and associated with lower mortality, largely due to greater prevalence of hypopigmented MF.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Asian People; Child; Child, Preschool; Female; Humans; Male; Middle Aged; Mycosis Fungoides; Prognosis; Retrospective Studies; Sezary Syndrome; Young Adult
PubMed: 30801779
DOI: 10.1111/jdv.15526 -
Dermatologic Clinics Oct 2015Primary cutaneous lymphomas (PCLs) are an extremely heterogeneous group of non-Hodgkin lymphomas that manifest in the skin. Their diagnosis is complex and based on... (Review)
Review
Primary cutaneous lymphomas (PCLs) are an extremely heterogeneous group of non-Hodgkin lymphomas that manifest in the skin. Their diagnosis is complex and based on clinical lesion type and evaluation of findings on light microscopic examination, immunohistochemistry and molecular analysis of representative skin biopsies. The evaluation, classification, and staging system is unique for mycosis fungoides (MF) and Sézary syndrome (SS), the most common subtypes of cutaneous T-cell lymphoma (CTCL) versus the other subtypes of Non-MF/Non-SS CTCL and the subtypes of cutaneous B-cell lymphoma (CBCL). Since current treatment is stage-based, it is particularly important that the correct diagnosis and stage be ascertained initially. The purpose of this article is to review the current evaluation, diagnosis, classification, staging, assessment techniques, and response criteria for the various types of both T-cell and B-cell PCLs.
Topics: Humans; Lymphoma, B-Cell; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Neoplasm Staging; Prognosis; Sezary Syndrome; Skin Neoplasms
PubMed: 26433839
DOI: 10.1016/j.det.2015.06.001 -
Leukemia & Lymphoma Mar 2018Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common cutaneous T-cell lymphomas (CTCLs). Both lack curative options, and advanced-stage carries a poor... (Review)
Review
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common cutaneous T-cell lymphomas (CTCLs). Both lack curative options, and advanced-stage carries a poor prognosis. Whilst there are a number of treatments available, achieving and maintaining a durable remission remains challenging. We review current systemic treatment options as monotherapy for advanced-stage MF (IIB-IV), appraising their mechanism of action, analyzing their efficacy, and describing toxicities. Individually, reported overall response rates (ORR) vary widely in the literature and duration of responses are typically short, ranging from 7.5 to 22.4 months. Combined therapy is frequently used in an effort to boost responses, although prospective studies comparing combinations to single agent therapies are rarely conducted. While recent translational research has led to increased understanding of the immunopathogenesis of MF and SS and the development of new treatments, current standard of care therapies are not curative and have low ORR for advanced-stage disease.
Topics: Antineoplastic Agents; Humans; Mycosis Fungoides; Prognosis; Sezary Syndrome; Skin Neoplasms
PubMed: 29308723
DOI: 10.1080/10428194.2017.1347650 -
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