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1.
Kathmandu Univ Med J (KUMJ) ; 22(86): 154-159, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39328103

RESUMEN

Background Primary cutaneous lymphomas (PCLs) are rare diagnoses in Nepal and are not well characterized till date. Objective To evaluate clinical and pathological features of Primary cutaneous lymphomas in Nepal. Method We retrospectively reviewed outpatient and inpatient records of a dermatology referral centre of Kathmandu, Nepal for clinical and pathological findings of cases diagnosed as cutaneous lymphomas from July 2010 through July 2020. The final diagnosis was made based on 2008 World Health Organization classification and its update 2018. Result There were 12 cases of Primary cutaneous lymphomas diagnosed during this period. The age of presentation ranged from 19 years to 81 years (Mean: 53.4 years ± 21.5 years, SD). There were ten cases of cutaneous T-cell lymphoma (CTCLs) and two cases of cutaneous B- cell lymphomas (CBCLs). Among cutaneous T-cell lymphoma, there were four cases of primary cutaneous anaplastic large- cell Lymphoma (PCALCL), two cases of classic (patch/plaque) mycosis fungoides (MF), two cases of folliculotropic mycosis fungoides (FMF), and one case each of primary cutaneous aggressive epidermotropicCD8+ T-cell lymphoma and lymphomatoid papulosis. Among cutaneous B- cell lymphomas, there was one case of primary cutaneous marginal zone B- cell lymphoma, and one case of primary cutaneous follicle centre lymphoma. Most cases of MF presented at stage IB (75%), and three patients of primary cutaneous lymphomas died during this period. Conclusion Primary cutaneous lymphomas appear to be very rare in this study and presentations ranged from classic Mycoses Fungoides to aggressive T-cell lymphomas. Cutaneous T-cell lymphomas appeared to be more common than cutaneous B- cell lymphomas in this study.


Asunto(s)
Linfoma Cutáneo de Células T , Neoplasias Cutáneas , Humanos , Nepal/epidemiología , Persona de Mediana Edad , Estudios Retrospectivos , Masculino , Femenino , Adulto , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/epidemiología , Anciano , Anciano de 80 o más Años , Linfoma Cutáneo de Células T/patología , Linfoma Cutáneo de Células T/epidemiología , Adulto Joven , Estudios de Cohortes , Linfoma de Células B/epidemiología , Linfoma de Células B/patología , Dermatología , Micosis Fungoide/patología , Micosis Fungoide/epidemiología
2.
Sci Rep ; 14(1): 20118, 2024 08 29.
Artículo en Inglés | MEDLINE | ID: mdl-39210040

RESUMEN

The relative frequency of primary cutaneous lymphoma (PCL) subtypes shows wide variation across different geographical regions. This retrospective study was conducted in a tertiary referral center located in Korea to describe the relative frequency, demographics, survival outcomes, and temporal trend in PCL. A total of 627 PCL cases diagnosed between January 1994 and December 2022 were included. The majority of PCL cases (87.2%) were of T-/NK-cell lineage (CTCL), while the remaining cases (12.8%) were B-cell lineage lymphomas (CBCL). The prevalence of mycosis fungoides (MF) in CTCL increased significantly over time, while other CTCL subtypes, including primary cutaneous extranodal NK/T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma (SPTCL), decreased in frequency. Notably, the prevalence of CD4-positive small/medium T-cell lymphoproliferative disorder showed a substantial increase over time. Primary cutaneous marginal zone lymphoma was consistently the commonest CBCL subtype. Survival analysis demonstrated that CTCL had a more favorable 5-year overall survival (OS) than CBCL. OS rate of MF, SPTCL, and primary cutaneous peripheral T-cell lymphoma, NOS improved significantly over time. This study provides comprehensive insights into the dynamic change in the relative frequency and overall survival of PCL subtypes over time.


Asunto(s)
Neoplasias Cutáneas , Centros de Atención Terciaria , Humanos , Masculino , Femenino , Estudios Retrospectivos , República de Corea/epidemiología , Persona de Mediana Edad , Neoplasias Cutáneas/mortalidad , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/epidemiología , Prevalencia , Adulto , Anciano , Linfoma Cutáneo de Células T/mortalidad , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/patología , Adulto Joven , Anciano de 80 o más Años , Adolescente , Linfoma de Células B/mortalidad , Linfoma de Células B/epidemiología , Linfoma de Células B/patología , Niño , Análisis de Supervivencia
3.
J Nepal Health Res Counc ; 22(1): 123-129, 2024 Jun 22.
Artículo en Inglés | MEDLINE | ID: mdl-39080948

RESUMEN

BACKGROUND: Primary cutaneous lymphomas are a distinct group of rare lymphoid neoplasms with absence of extracutaneous lymphomas at the time of presentation. They are rare in Nepal and no data on cutaneous lymphoma have been published from this country till date. METHODS: This retrospective study included 15 cases of cutaneous lymphomas retrieved from the records of department of Dermatopathology, DI Skin Hospital and Referral Centre, Bansbari, Kathmandu, Nepal. Patients were diagnosed according to the current WHO classification for cutaneous lymphoma. RESULTS: A total of 15 cases were studied with median age of 45 years (range: 22 to 81 years) and male to female ratio of 1.5:1. Primary cutaneous lymphomas constituted 13 cases out of 15 and the most common type of cutaneous lymphoma was mycosis fungoides and variants 5 (33%), followed by CD30 positive primary cutaneous anaplastic large cell lymphoma constituting 2 (13%). T-cell cutaneous lymphoma constituted 13 (87%) and B-cell cutaneous lymphoma 2 (13%). CONCLUSIONS: Cutaneous T-cell lymphomas were more frequent than cutaneous B-cell lymphomas in Nepalese patients. Mycosis fungoides and variants are commonest type of primary cutaneous lymphomas.


Asunto(s)
Neoplasias Cutáneas , Centros de Atención Terciaria , Humanos , Nepal/epidemiología , Masculino , Femenino , Persona de Mediana Edad , Adulto , Estudios Retrospectivos , Anciano , Anciano de 80 o más Años , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/patología , Adulto Joven , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/patología , Micosis Fungoide/epidemiología , Micosis Fungoide/patología , Linfoma de Células B/epidemiología , Linfoma de Células B/patología
5.
J Am Acad Dermatol ; 91(2): 255-258, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38588818

RESUMEN

BACKGROUND: Dupilumab, a human monoclonal antibody targeting the interleukin 4 alpha receptor, is used for treatment of moderate to severe atopic dermatitis (AD). Previous studies have reported diagnoses of cutaneous T cell lymphoma (CTCL) after dupilumab use. OBJECTIVE: Investigate the risk of CTCL after dupilumab use in patients with AD. METHODS: Using the TrinetX database, incidence of cutaneous and lymphoid malignancies including CTCL was compared between a cohort of patients with AD who used dupilumab and a cohort of patients with AD who never used dupilumab. A second analysis excluding prior disease-modifying antirheumatic drug use was performed. Propensity score matching was performed to control for covariates. RESULTS: An increased risk of CTCL was found in the cohort of AD patients who used dupilumab (odds ratio 4.1003, 95% confidence interval 2.055-8.192). The increased risk persisted after exclusion of prior disease-modifying antirheumatic drug use. Risk was not increased for other cutaneous or lymphoid malignancies. Most (27/41) cases of CTCL were diagnosed more than 1 year after dupilumab use. LIMITATIONS: There is potential for misclassification in the database. Severity of AD could not be assessed. Association between dupilumab and CTCL does not prove causality. CONCLUSION: Dupilumab use is associated with an increased risk of CTCL in patients with AD in this cohort.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Dermatitis Atópica , Linfoma Cutáneo de Células T , Neoplasias Cutáneas , Humanos , Dermatitis Atópica/tratamiento farmacológico , Dermatitis Atópica/epidemiología , Anticuerpos Monoclonales Humanizados/efectos adversos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Masculino , Estudios Retrospectivos , Femenino , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/epidemiología , Adulto , Persona de Mediana Edad , Linfoma Cutáneo de Células T/tratamiento farmacológico , Linfoma Cutáneo de Células T/epidemiología , Incidencia , Medición de Riesgo/estadística & datos numéricos , Anciano , Puntaje de Propensión
6.
Arch Dermatol Res ; 316(2): 74, 2024 Jan 18.
Artículo en Inglés | MEDLINE | ID: mdl-38236413

RESUMEN

Cutaneous T-cell lymphomas (CTCL) are a rare group of T-cell neoplasms which infiltrate the skin and can result in substantial morbidity and mortality. Risk factors for CTCL are still poorly understood though recent studies suggest chemical exposures may play a role in its development. To further characterize patient-centered risk factors for CTCL, especially compared with matched controls, we performed one of the largest prospective cohort survey studies to date to examine patient-reported exposures and health-related quality of life (HRQoL) in association with concurrent clinical disease characteristics. Patient demographics, lifestyle factors, and chemical exposures were collected via clinical data and surveys. Descriptive statistics, ANOVA, Chi-square tests and t tests were utilized to compare patient-reported exposures and HRQoL in patients with CTCL versus matched healthy controls (HC). Statistically significant differences were identified between patients and HC in terms of race (non-white race 22.4% in CTCL patients vs. 18.8% in HC, P = 0.01), and education level (high school or less 41.6% in CTCL patients vs. 14.3% in HC, P = 0.001), but not with Fitzpatrick skin type (P = 0.11) or smoking status (P = 0.28). Notably, 36.0% of the CTCL patients reported exposures to chemicals, a near threefold increased percentage when compared to HC (12.9%). Among various chemical exposures, 27.0% of the CTCL patients specifically reported industrial chemical exposure, a more than two-fold increased percentage when compared to HC (12.9%). Itch and pain were significantly associated with skin disease severity (as evaluated by CTCL-specific mSWAT score) in advanced stage disease (stages IIB-IVB) (r = 0.48 and 0.57, P < 0.05). Itch and body mass index (BMI) were weakly associated with skin disease severity in early-stage disease (stages IA-IIA) (r = 0.27 and 0.20, P < 0.05).


Asunto(s)
Linfoma Cutáneo de Células T , Médicos , Neoplasias Cutáneas , Humanos , Estudios de Cohortes , Estudios Prospectivos , Calidad de Vida , Linfoma Cutáneo de Células T/epidemiología , Prurito , Neoplasias Cutáneas/epidemiología
9.
Actas Dermosifiliogr ; 115(2): 119-129, 2024 Feb.
Artículo en Inglés, Español | MEDLINE | ID: mdl-37689349

RESUMEN

BACKGROUND AND OBJECTIVE: The cost of treating cutaneous T-cell lymphoma (CTCL) in Spain is unknown. With the advent of new treatments, it is more important than ever to gain an accurate picture of the true costs involved. The MICADOS study had 2 primary objectives: 1)to evaluate the impact of CTCL on patient quality of life, and 2)to evaluate the costs associated with the disease. This article reports the results of the cost analysis. METHODS: We estimated the cost of treating CTCL over a period of 1year from the perspective of the Spanish National Health System. Twenty-three dermatologists and hematologists from 15 public hospitals analyzed data for adult patients with mycosis fungoides (MF) or Sézary syndrome (SS). RESULTS: A total of 141 patients (57.4% male) with a mean age of 63.6 years (95%CI: 61.4-65.7 years) were included. The mean direct annual cost of treating CTCL was €34,214 per patient. The corresponding costs by stage were €11,952.47 for stageI disease, €23,506.21 for stageII disease, €38,771.81 for stageIII disease, and €72,748.84 for stageIV disease. The total direct annual cost of treating MF/SS in public hospitals in Spain was estimated at €78,301,171; stageI disease accounted for 81% of all costs, stageII for 7%, and stagesIII andIV for 6% each. CONCLUSIONS: The MICADOS study offers an accurate picture of the direct cost of treating CTCL in patients with MF/SS in Spain and shows that costs vary significantly according to disease stage. Patient-borne and indirect costs should be analyzed in future studies.


Asunto(s)
Linfoma Cutáneo de Células T , Micosis Fungoide , Síndrome de Sézary , Neoplasias Cutáneas , Adulto , Humanos , Masculino , Persona de Mediana Edad , Femenino , Calidad de Vida , España/epidemiología , Neoplasias Cutáneas/terapia , Neoplasias Cutáneas/patología , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/terapia , Linfoma Cutáneo de Células T/patología , Micosis Fungoide/terapia , Micosis Fungoide/patología , Síndrome de Sézary/terapia , Síndrome de Sézary/patología
10.
Ital J Dermatol Venerol ; 158(6): 467-482, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-38015484

RESUMEN

INTRODUCTION: Cutaneous T-cell lymphoma (CTCL) is a heterogenous group of non-Hodgkin lymphomas. Similar presentation to benign conditions, significant genetic variation, and lack of definitive biomarkers contributes to diagnostic delay. The etiology of CTCL is unknown, and environmental exposures, such as geographic, occupational, chemicals, sunlight, and insects have been investigated. EVIDENCE ACQUISITION: Review of the literature for CTCL and exposures was performed in PubMed and Google Scholar in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) Extension for Scoping Reviews. This search yielded 193 total results, which were initially screened with defined inclusion and exclusion criteria. The 45 remaining articles were reviewed and classified by exposure type. EVIDENCE SYNTHESIS: The most frequently investigated CTCL exposure type was geographic (13/45 articles, 29%). Chemical exposures were commonly discussed (10/45 articles, 22%), along with occupational (10/45 articles, 22%). Insect exposures (6/45, 13%) and sun exposure (3/45, 7%) were also reviewed, along with articles describing multiple exposure types (3/45, 7%). Article types ranged from cases to systematic reviews and case-control studies. Evidence linking CTCL and these exposures was mixed. Limitations of this investigation include reliance on patient reporting and frequent speculation on disease association versus causality. CONCLUSIONS: This investigation synthesizes the current literature on exposures potentially implicated in the pathogenesis of CTCL, while offering guidance on patient history-taking to ensure potential exposures are captured. Awareness of these possible associations may improve understanding of disease pathogenesis and diagnosis. Moreover, these insights may help with public health decision-making and disease mitigation.


Asunto(s)
Linfoma no Hodgkin , Linfoma Cutáneo de Células T , Neoplasias Cutáneas , Humanos , Diagnóstico Tardío , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/etiología , Exposición a Riesgos Ambientales/efectos adversos , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/etiología
11.
Eur J Dermatol ; 33(4): 360-367, 2023 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-37823487

RESUMEN

Among primary cutaneous T-cell lymphomas (CTCL), mycosis fungoides (MF) is the most frequent and, along with Sézary syndrome (SS), the best-studied subtype. Most available studies on epidemiology of MF and SS are based on small cohorts or different inclusion criteria. Moreover, although this has become a hot topic, most studies show limitations, such as selection bias and lack of clinical information or follow-up data. Therefore, no reliable conclusions can be drawn. This paper reviews the current data underpinning our understanding of the epidemiology of MF and SS, and presents some original findings based on data retrieved from the cutaneous lymphoma registry of the Italian Marche region. The Marche Regional Cutaneous Lymphoma Registry is a multidisciplinary team founded 27 years ago to share the management of these rare disorders. All patients with a clinical and histologically confirmed diagnosis of primary cutaneous lymphoma are centralized in Ancona (Italy) at the Haematology Clinic, Polytechnic University of Marche, for clinical evaluation, staging, treatment, and follow-up. This paper emphasizes the need for a national registry of pCLs in Italy, as no detailed epidemiological information is available in the country except for the Marche Regional Cutaneous Lymphoma Registry. A national registry would allow for more comprehensive data collection from all over Italy and could provide more accurate information on incidence and epidemiology. This would be beneficial for understanding the pathogenesis and diagnostic procedures of these diseases and could improve patient outcomes. Therefore, we advise the creation of a national registry of pCLs in Italy.


Asunto(s)
Linfoma Cutáneo de Células T , Linfoma , Micosis Fungoide , Síndrome de Sézary , Neoplasias Cutáneas , Humanos , Micosis Fungoide/patología , Síndrome de Sézary/patología , Neoplasias Cutáneas/patología , Italia/epidemiología , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/terapia , Linfoma Cutáneo de Células T/patología
12.
J Dtsch Dermatol Ges ; 21(11): 1320-1327, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37845021

RESUMEN

BACKGROUND: Primary cutaneous lymphomas (PCL) are rare skin tumors of lymphoproliferative neoplasms and belong to the heterogeneous group of non-Hodgkin's lymphomas. PCL encompass a broad spectrum of clinical and histologic manifestations, with cutaneous T-cell lymphoma (CTCL) being the most common (73%). Due to the rarity of the diseases, population-based studies of care and epidemiology are limited. PATIENTS AND METHODS: Based on anonymized, age- and sex-adjusted SHI (statutory health insurance) claims data of approximately five million SHI-insured patients, a retrospective analysis was conducted over a six-year period (2012-2017) to determine the prevalence, incidence, and lethality in patients with mature-cell T/NK-cell lymphoma in Germany. RESULTS: A total of 1,336 patients with T-cell lymphoma were identified during the observation period. The six-year prevalence ranged from 27.35 to 43.58 per 100,000. Patients were 65% male with a mean age of 66 years (SD 15). There were 246 patients (approx. 20%) who died within the 6 years, up to 7% per year. The calculated incidence in 153 identified patients in 2017 is 3.65 to 3.92 per 100,000. CONCLUSIONS: For the first time, valid epidemiologic findings of patients with mature T-cell and NK-cell lymphomas were obtained using SHI claims data in Germany. Further analyses are needed to gain a deeper insight into the healthcare reality of patients with this rare disease.


Asunto(s)
Linfoma Cutáneo de Células T , Micosis Fungoide , Síndrome de Sézary , Neoplasias Cutáneas , Humanos , Masculino , Anciano , Femenino , Estudios Transversales , Estudios Retrospectivos , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/patología , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/patología , Alemania/epidemiología , Micosis Fungoide/patología
13.
Clin Lymphoma Myeloma Leuk ; 23(12): 897-904, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37690902

RESUMEN

BACKGROUND: Gamma delta T cells gives rise to a rare malignancy called Primary cutaneous Gamma-Delta T cell lymphoma (PCGDTCL). METHODS: From the National Cancer Database (NCDB), 110 (0.015%) patients with PCGDTCL were identified. RESULTS: Males aged >60 years were the commonest cohort. Caucasian race was the most common (Caucasian: 79.09%, African American:16.36%). Most patients were diagnosed at stage 1 (52.33%), followed by stage 4 (30.23%). On analyzing income categories, <$48,000 group had 48.15% stage 4 (13/27) and 40.74% (11/27) stage 1. Overall survival (OS) of the study group at 3 years by Kaplan-Meier (KM) analysis was 46.6%. African American race (37.5%), income of <$48,000 (27.6%) and government insurance (38.8%) had lower survival rates in KM analysis. In the adjusted hazard ratio (HR) analysis, only age <=40 years compared to >60 years (0.165 [0.036, 0.768], P= .0217) reached significance. Although the group that did not receive any chemotherapy or radiation seemed to have a better survival by KM analysis at 74.3% at 3 years, significance was not seen in the adjusted HR estimates and majority of the patients in this group were stage 1. This group may have received topical treatments which may have not been captured in NCDB. Adjusted analysis also revealed chemoradiation to have a lower mortality risk compared to chemotherapy alone (0.229 [0.079, 0.670], P = .0071), suggesting that aggressive strategies may be required for management when needed. CONCLUSION: Socioeconomic disparities significantly impact access to healthcare and are of particular importance in rare lymphomas.


Asunto(s)
Linfoma Cutáneo de Células T , Linfoma de Células T , Linfoma , Neoplasias Cutáneas , Masculino , Humanos , Estudios Retrospectivos , Neoplasias Cutáneas/patología , Linfocitos T , Linfoma/patología , Linfoma de Células T/patología , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/terapia
15.
Clin Exp Dermatol ; 48(9): 1036-1039, 2023 Aug 25.
Artículo en Inglés | MEDLINE | ID: mdl-37140442

RESUMEN

Primary cutaneous lymphomas (PCLs) are non-Hodgkin lymphomas, presenting in the skin. They are classified into cutaneous B-cell lymphomas (CBCLs) or cutaneous T-cell lymphomas (CTCLs). The most common CTCL subtypes are mycosis fungoides (MF) and Sézary syndrome (SS). All patient's cases should be discussed at a specialist multidisciplinary team (MDT) meeting. This is the first published review, to our knowledge, of PCL MDT case discussion in the UK. Patient cases between 2008 and 2019 were reviewed to assess: frequency of PCL subtype, documentation of CTCL staging and management of MF/SS. Of 356, 103 (29%) were CBCLs and the majority (n = 200, 56%) were CTCLs. MF/SS was the diagnosis in 120 (34%). Staging was documented in 44% (n = 53) of patients with MF/SS. Management largely followed guidelines for MF/SS, topical corticosteroids were the most common treatment (n = 93, 78%). Frequency of PCL subtype is largely comparable with previously published data. Documentation of CTCL staging is low, but higher than other reports. Our work begins to address the gap in real-world data on CTCLs. A standardized approach to data collection would inform clinical practice.


Asunto(s)
Linfoma de Células B , Linfoma Cutáneo de Células T , Micosis Fungoide , Síndrome de Sézary , Neoplasias Cutáneas , Humanos , Micosis Fungoide/diagnóstico , Micosis Fungoide/terapia , Micosis Fungoide/patología , Linfoma Cutáneo de Células T/diagnóstico , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/terapia , Síndrome de Sézary/diagnóstico , Síndrome de Sézary/terapia , Síndrome de Sézary/patología , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/terapia , Neoplasias Cutáneas/patología , Linfoma de Células B/patología , Reino Unido/epidemiología
16.
Dermatology ; 239(4): 553-564, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37231916

RESUMEN

BACKGROUND: The incidence and clinical features of primary cutaneous lymphoma (PCL) tend to differ by age, gender, geographical, and racial variation. All-aged and adult groups of PCL in various regions have been well demonstrated and compared, while the research concentrating on pediatric PCL is rare, especially in Asian countries. OBJECTIVE: The aim of this study was to investigate the clinical characteristics of PCL in pediatric population at a single center in China. METHODS: We conducted a retrospective study of 101 pediatric cases with PCL, diagnosed at the Institute of Dermatology, Chinese Academy of Medical Sciences, from January 2010 to December 2021. RESULTS: Mycosis fungoides (MF), accounting for 41.6% of the total cases, was the most common subtype in pediatric PCL, and the hypopigmented MF accounted for 47.6% of all the MF cases. Lymphomatoid papulosis and chronic active Epstein-Barr virus infection tied for second place with a proportion of 22.8%. Primary cutaneous anaplastic large cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, primary cutaneous peripheral T-cell lymphoma, rare subtypes and primary cutaneous B-cell lymphoma, respectively, accounted for 2.0%, 4.0%, 4.0%, and 3.0%. Most patients had favorable prognosis during the follow-up. CONCLUSION: The study suggested that MF was the most common subtype in pediatric PCL in China, and most types of pediatric PCL had favorable prognosis.


Asunto(s)
Infecciones por Virus de Epstein-Barr , Linfoma Cutáneo de Células T , Micosis Fungoide , Neoplasias Cutáneas , Adulto , Humanos , Niño , Anciano , Linfoma Cutáneo de Células T/diagnóstico , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/patología , Estudios Retrospectivos , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/patología , Herpesvirus Humano 4 , Micosis Fungoide/diagnóstico , Micosis Fungoide/epidemiología , Micosis Fungoide/patología , China/epidemiología
17.
JAMA Dermatol ; 159(2): 192-197, 2023 02 01.
Artículo en Inglés | MEDLINE | ID: mdl-36598766

RESUMEN

Importance: Cutaneous T-cell lymphoma (CTCL) is a group of rare, complex cutaneous malignant neoplasms associated with significant disease burden on patients and the health care system. Currently, the population of patients with CTCL admitted to the hospital remains largely uncharacterized and poorly understood. Objective: To characterize the clinical characteristics, course of hospitalization, and mortality outcomes of an inpatient CTCL cohort. Design, Setting, and Participants: This multicenter retrospective cohort study reviewed medical records for adult patients (age ≥18 years) with a CTCL diagnosis per National Comprehensive Cancer Network guidelines admitted for inpatient hospitalization at 5 US academic medical centers with inpatient dermatology consult services and CTCL clinics between August 2016 and August 2020. Main Outcomes and Measures: Patient demographics, clinical history and findings, hospitalization courses, and mortality outcomes. Results: A total of 79 hospitalized patients with CTCL were identified, including 52 (70.3%) men and 22 (29.7%) women, with a median (IQR) age at hospitalization of 62.9 (27-92) years. The majority of admitted patients with CTCL were White (65 patients [82.3%]), had disease classified as mycosis fungoides (48 patients [61.5%]), and had advanced-stage disease (≥IIB, 70 patients [89.7%]). Most hospitalizations were complicated by infection (45 patients [57.0%]) and required intravenous antibiotic therapy (45 patients [57.0%]). In-hospital mortality occurred in 6 patients (7.6%) and was associated with higher body mass index (36.5 vs 25.3), history of thromboembolic disease (50.0% vs 12.3%), and diagnosis of sepsis on admission (66.7% vs 20.5%). At 1-year postdischarge, 36 patients (49.3%) patients had died, and mortality was associated with history of solid organ cancers (27.8% vs 10.8%), wound care as the reason for dermatology consultation (58.3% vs 24.3%), and presence of large cell transformation (58.3% vs 22.9%). Conclusions and Relevance: The findings of this cohort study improve the understanding of hospitalized patients with CTCL and lend valuable insight into identifying factors associated with both in-hospital and long-term mortality outcomes. This refined understanding of the inpatient CTCL population provides a foundation for larger, more robust studies to identify causal risk factors associated with mortality, development of prognostic scoring systems to estimate the probability of hospital mortality. Overall, the findings may prompt physicians caring for patients with CTCL to implement preventive strategies to diminish hospitalization and improve clinical management across this unique disease spectrum.


Asunto(s)
Linfoma Cutáneo de Células T , Neoplasias Cutáneas , Adulto , Masculino , Humanos , Femenino , Adolescente , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Estudios de Cohortes , Estudios Retrospectivos , Cuidados Posteriores , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/terapia , Alta del Paciente , Linfoma Cutáneo de Células T/diagnóstico , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/terapia
19.
Actas Dermosifiliogr ; 114(4): 291-298, 2023 Apr.
Artículo en Inglés, Español | MEDLINE | ID: mdl-36529273

RESUMEN

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.


Asunto(s)
Dermatología , Linfoma Cutáneo de Células T , Micosis Fungoide , Neoplasias Cutáneas , Venereología , Masculino , Humanos , Persona de Mediana Edad , Femenino , Linfoma Cutáneo de Células T/diagnóstico , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/terapia , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/terapia , Sistema de Registros , Micosis Fungoide/patología
20.
Arch Dermatol Res ; 315(2): 275-278, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-35668201

RESUMEN

Cutaneous T-cell lymphoma (CTCL) risk factors and associated quality of life are poorly understood. Previous studies of CTCL risk factors explored patient comorbidities and lifestyle exposures, but not in conjunction with disease stage, subtype, severity, or health-related quality of life (HRQoL). We investigated lifestyle exposures and demographic factors associated with advanced-stage disease, increased disease severity, and poorer HRQoL outcomes in this single-center cohort study. A cohort survey study was conducted at Northwestern's Multidisciplinary Cutaneous Lymphoma specialty clinic between April 2019 and June 2021. REDCap surveys were administered to 140 patients with CTCL, investigating patients' demographics, lifestyle and chemical exposures. QoL was evaluated using the Skindex survey; pain and itch with ten-point Likert scales. Modified Severity Weighted Assessment Tool (mSWAT), disease stage, and disease subtype were confirmed upon enrollment in the study by a single board-certified dermatologist specializing in CTCL. Factors were compared by t test or Fischer's exact test. Median age was 63 years (range 14-92) with male-to-female ratio of 1.2:1. The most common diagnosis was CD4 + MF (n = 94, 67.1%). Common lifestyle exposures included smoking (past or current) (52.3%) and chemical exposure history (all sources [53.7%]; industrial only [33.0%]). History of chemical exposures were associated with advanced stage disease (p = 0.003) and worse QoL outcomes (p = 0.001). There were significant racial differences, respectively, in early (I-IIA) vs late (IIB-IV) stage disease (p = 0.003). Obesity, hygiene, smoking, recent sun exposure, education and atopy were not significantly associated with disease stage or severity. We provide an analysis of lifestyle and demographic factors in the context of CTCL disease severity, stage, and HRQoL. We identified race as a potential risk factor for advanced stage disease and both skin phototype and chemical exposures as a risk factor for increased disease severity as measured by mSWAT. QoL outcomes were multifactorial and significantly associated with history of chemical exposure, severe pain/itch, race, disease stage and subtype. An improved understanding of these associations may lead to better individualized care. As chemical exposure and race were found to be significant factors associated with advanced-stage disease, taking exposure histories and addressing racial disparities may improve care for CTCL patients.


Asunto(s)
Linfoma Cutáneo de Células T , Micosis Fungoide , Síndrome de Sézary , Neoplasias Cutáneas , Humanos , Masculino , Femenino , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Micosis Fungoide/patología , Calidad de Vida , Estudios de Cohortes , Linfoma Cutáneo de Células T/epidemiología , Linfoma Cutáneo de Células T/patología , Neoplasias Cutáneas/patología , Prurito , Estilo de Vida
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