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1.
J Eur Acad Dermatol Venereol ; 33(8): 1513-1521, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30801779

RESUMO

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.


Assuntos
Grupo com Ancestrais do Continente Asiático , Micose Fungoide/epidemiologia , Síndrome de Sézary/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/patologia , Micose Fungoide/terapia , Prognóstico , Estudos Retrospectivos , Síndrome de Sézary/patologia , Síndrome de Sézary/terapia , Adulto Jovem
2.
J Infect Dis ; 219(10): 1564-1573, 2019 04 19.
Artigo em Inglês | MEDLINE | ID: mdl-30357388

RESUMO

BACKGROUND: Human skin microorganisms have been associated with various skin diseases. However, most studies have focused on bacterial communities, and little is known about normally resident skin viruses such as the Polyomaviridae and their association with cutaneous disorders. METHODS: We investigated the infection levels of Merkel cell polyomavirus (MCPyV), human polyomavirus 6 (HPyV6), and human polyomavirus 7 (HPyV7), using triplet skin swabs collected from lesional and nonlesional skins of 86 Japanese patients with inflammatory skin diseases and mycosis fungoides, and from 149 healthy control individuals. RESULTS: This age-matched case-control study provides the first analyses of the loads of polyomaviruses in association with various skin diseases. The viral loads were significantly higher for HPyV6/HPyV7 and lower for MCPyV in patients with psoriasis. The viral load variation was observed not only at lesion sites, but also at clinically unaffected skin sites in most of the patients. The viral strains tested were all of the Asian/Japanese genotype. CONCLUSIONS: Our findings suggest a covariation in the infection levels of cutaneous polyomaviruses in certain inflammatory skin conditions. Worldwide prospective longitudinal studies are warranted to understand the influence of such alterations on the pathogenesis of inflammatory skin disorders.


Assuntos
Infecções por Polyomavirus/epidemiologia , Polyomavirus/isolamento & purificação , Dermatopatias/epidemiologia , Pele/virologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , DNA Viral/isolamento & purificação , Feminino , Humanos , Japão/epidemiologia , Masculino , Poliomavírus das Células de Merkel/isolamento & purificação , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Micose Fungoide/virologia , Prevalência , Psoríase/virologia , Dermatopatias/virologia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/virologia , Infecções Tumorais por Vírus/epidemiologia , Infecções Tumorais por Vírus/virologia , Carga Viral
4.
Int J Dermatol ; 58(4): 449-455, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30294921

RESUMO

BACKGROUND: The aim of this study was to determine the relative frequency of primary cutaneous lymphoma (PCL) in Argentina according to the new World Health Organization (WHO)-European Organization for the Research and Treatment of Cancer (EORTC) classification system. METHODS: A total of 416 patients from 21 dermatology services were included during a 5-year period (2010-2015); these patients were classified using WHO-EORTC criteria. RESULTS: There were 231 (55.2%) males and 185 (44.8%) females; the male-to-female ratio was 1.35. The median age of the patients was 57 years (range, 0-90 years). Most patients were Caucasian (79%), and only 16% of patients were registered as Amerindian. Most patients (387/416, 93%) had cutaneous T-cell lymphoma (CTCL); 28 patients (6.7%) were diagnosed with cutaneous B-cell lymphoma (CBCL). The most frequent CTCL subtypes, in decreasing order of prevalence, were mycosis fungoides (MF), including its variants (75.7%); CD30+ primary cutaneous lymphoproliferative disorders (7.2%); and Sézary syndrome (SS) (3.1%). Cutaneous follicle center lymphoma was the most common CBCL subtype (2.9%). In the subset of patients ≤20 years of age, the most common condition was MF (57%), followed by extranodal NK-T nasal-type lymphoma (14%). CONCLUSIONS: This study revealed relatively higher rates of MF and lower rates of CBCL in Argentinean patients that have been reported in American and European countries.


Assuntos
Linfoma de Células B/epidemiologia , Micose Fungoide/epidemiologia , Síndrome de Sézary/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Argentina/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Linfoma Extranodal de Células T-NK/epidemiologia , Linfoma Folicular/epidemiologia , Masculino , Pessoa de Meia-Idade , Adulto Jovem
5.
Pathology ; 50(7): 718-724, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30348505

RESUMO

Mycosis fungoides (MF) is an indolent cutaneous T-cell lymphoma and may transform into large cell lymphoma in the disease course. The incidence of MF in Taiwan is lower as compared to that in the West. In this study we aimed to characterise the clinicopathological, immunohistochemical, and genetic features of transformed MF (t-MF) in Taiwan. We retrospectively collected MF cases from April 2004 to April 2015 from four medical centres in Taiwan, reviewed the clinical history and histopathology, and performed immunohistochemistry, in situ hybridisation for EBV (EBER), and fluorescence in situ hybridisation (FISH) for DUSP22/MUM1 gene translocation. Fifty-one specimens from 32 patients with MF were identified with a male to female ratio of 1.5:1 and a median age of 50.5 (range 16-82). Tumours from 11 patients (34%) underwent large cell transformation, with the median age at 61 (range 26-82). The tumour cells of t-MF expressed CD30 and MUM1 in 82% and 100% cases, respectively. CD56 was expressed in two (10%) of 21 MF cases and two (18%) of 11 t-MF cases, respectively; and all four CD56-positive cases were of a helper T-cell phenotype. All CD56 expressing MF and t-MF tumours tested for EBER were negative. FISH study showed rearranged DUSP22/IRF4 in one (9%) of 11 t-MF cases, but not in any of the 19 non-transformed MF specimens. Four patients with t-MF died of disease and six were alive with disease in a median follow-up time of 25 months (mean 44.7 months). Large cell transformation and aberrant CD56 expression were more frequent in patients with MF in Taiwan compared to those in the West. Larger case series and/or national studies are needed to clarify the significance and impact of large cell transformation on the prognosis of patients with MF.


Assuntos
Antígeno CD56/metabolismo , Fosfatases de Especificidade Dupla/genética , Fatores Reguladores de Interferon/genética , Fosfatases da Proteína Quinase Ativada por Mitógeno/genética , Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Transformação Celular Neoplásica , Feminino , Rearranjo Gênico , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Masculino , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Micose Fungoide/genética , Estudos Retrospectivos , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/genética , Taiwan/epidemiologia , Adulto Jovem
6.
Saudi Med J ; 39(10): 994-998, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30284581

RESUMO

OBJECTIVES: To determine the histopathological pattern of skin cancer in Madinah region of Kingdom of Saudi Arabia (KSA). Methods: This is a retrospective study including all cases with a histological diagnosis of skin cancer diagnosed at the Department of Pathology, King Fahad Hospital, Madinah, Saudi Arabia, between January 2006 and December 2017. Data included  age, gender, site and histology of the tumor were collected from histopathological reports and analyzed using Statistical Package for the Social Sciences  (SPSS) Version 21. Results: Among the 202 cancer cases studied in our series, there were 124 (61.4%) cases of basal cell carcinoma (BCC), 33 (16.3%) cases of squamous cell carcinoma (SCC), 14 (6.8%) cases of mycosis fungoides (MF), 12 (5.8%) cases of dermatofibrosarcoma protuberans (DFSP) and 7 (3.7%) cases of malignant melanomas (MM). The male to female ratio for all cases was 2.2:1. The ages ranged from 5 to 100 years with a mean age of 60.1±15 years. The peak age distribution was in the 60-69 year group. The most common site involved in BCC and SCC was the head and neck (92.7% and 66.7%) respectively). Acral distribution of MM was seen in 57.1% of the cases. Conclusion: Our data based on the histopathology history in the Madinah region match that in the world literature and Saudi national studies.


Assuntos
Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Basocelular/epidemiologia , Carcinoma Basocelular/patologia , Carcinoma de Células Escamosas/epidemiologia , Carcinoma de Células Escamosas/patologia , Criança , Pré-Escolar , Dermatofibrossarcoma/epidemiologia , Dermatofibrossarcoma/patologia , Feminino , Humanos , Incidência , Masculino , Melanoma/epidemiologia , Melanoma/patologia , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Micose Fungoide/patologia , Estudos Retrospectivos , Arábia Saudita/epidemiologia , Adulto Jovem
8.
An Bras Dermatol ; 93(4): 546-552, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30066762

RESUMO

BACKGROUND: Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. OBJECTIVE: The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. METHODS: This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. RESULTS: One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. STUDY LIMITATIONS: Small sample and retrospective design. CONCLUSIONS: The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.


Assuntos
Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Brasil/epidemiologia , Criança , Estudos Transversais , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Estadiamento de Neoplasias , Prevalência , Estudos Retrospectivos , Neoplasias Cutâneas/epidemiologia , Adulto Jovem
9.
Br J Dermatol ; 179(6): 1322-1328, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30098016

RESUMO

BACKGROUND: Infections are one of the major causes of death in patients with advanced-stage mycosis fungoides (MF) or Sézary syndrome (SS). However, few recent data are available on the characteristics and risk factors of these infectious events. OBJECTIVES: To describe infectious events occurring in a cohort of patients with MF/SS, and to identify associated clinical and biological risk factors. METHODS: A retrospective cohort study was performed to investigate infectious events and associated factors in patients diagnosed with MF (stage IB and beyond) or SS followed from May 2011 to May 2016 at the University Hospital of Bordeaux, France. RESULTS: Seventy-one patients with complete follow-up were included. Eighty infectious events were recorded in 40 patients, including 28 skin and soft tissue infections and 25 cases of pneumonia. Opportunistic infections, which are usually associated with depleted cell-mediated immunity, were scarce (9%). In multivariate analysis, cardiac, renal or lung comorbidities [odds ratio (OR) 7·2, 95% confidence interval (CI) 3·3-15·9; P = 0·002], SS (OR 8·8, 95% CI 7·7-10·2; P = 0·037) and lymphocyte count < 0·5 × 109 cells L-1 (OR 6·4, 95% CI 1·5-27·4; P = 0·004) were significantly associated with a higher risk of infection. CONCLUSIONS: Opportunistic germs were rarely recorded, but their incidence was probably prevented by adequate prophylaxis (ongoing in 28% of patients). As in patients living with AIDS, pneumonias were frequent. On the other hand, bacterial cutaneous infections represent a specific pattern in patients with MF/SS. Patients with chronic organ failure, lymphocytopenia and SS should be considered as being at high risk for infectious events. Pneumococcal vaccination should be systematically recommended, and prophylaxis with co-trimoxazole and valaciclovir when the CD4 count is < 0·2 × 109 cells L-1 .


Assuntos
Micose Fungoide/complicações , Infecções Oportunistas/epidemiologia , Pneumonia/epidemiologia , Síndrome de Sézary/complicações , Dermatopatias Infecciosas/epidemiologia , Neoplasias Cutâneas/complicações , Comorbidade , Feminino , Seguimentos , França/epidemiologia , Humanos , Incidência , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/sangue , Micose Fungoide/epidemiologia , Micose Fungoide/imunologia , Estadiamento de Neoplasias , Infecções Oportunistas/imunologia , Pneumonia/imunologia , Estudos Retrospectivos , Fatores de Risco , Síndrome de Sézary/sangue , Síndrome de Sézary/epidemiologia , Síndrome de Sézary/imunologia , Dermatopatias Infecciosas/imunologia , Neoplasias Cutâneas/sangue , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/imunologia
10.
An. bras. dermatol ; 93(4): 546-552, July-Aug. 2018. tab
Artigo em Inglês | LILACS | ID: biblio-949907

RESUMO

Abstract: Background: Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. Objective: The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. Methods: This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. Results: One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. Study limitations: Small sample and retrospective design. Conclusions: The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Neoplasias Cutâneas/patologia , Micose Fungoide/patologia , Neoplasias Cutâneas/epidemiologia , Brasil/epidemiologia , Criança , Prevalência , Estudos Transversais , Estudos Retrospectivos , Micose Fungoide/epidemiologia , Progressão da Doença , Estadiamento de Neoplasias
11.
Hautarzt ; 69(12): 1014-1020, 2018 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-29881891

RESUMO

BACKGROUND: In addition to a broad and clinically diverse spectrum of known primary cutaneous lymphomas, for which an incidence of 1-3:100,000 is postulated, each year further entities are specified and defined. The goal is the presentation of a case series from daily clinical routine. METHODS: Over a period of 6 years and 2 months, patients consulting the Department of Dermatology, Medical Center University of Freiburg, were registered. Subsequently, collectives of mycosis fungoides (MF), Sezary syndrome (SS), CD30+ lymphoproliferative diseases, single cases with rare primary cutaneous lymphomas, and subcollectives of B­cell lymphomas were examined. The high number of MF cases allowed the additional quantitative analyses of the types of therapies used in this group. RESULTS: Yearly 16-25 new diagnoses of primary cutaneous lymphoma are made. The evaluation of 163 primary cutaneous lymphoma revealed 111 cases with MF (68.1%), including 9 particular variants, 15 primary cutaneous CD30+ lymphoproliferative diseases (9.2%) dominated by 10 lymphomatoid papulosis (LyP), in addition to 5 primary cutaneous anaplastic large cell lymphoma (PCALCL), 6 SS (3.68%), and 24 cutaneous B­cell lymphomas (14-72%). Three cases with rare primary cutaneous T/NK cell lymphomas are addressed in detail. In all, 82% of MF cases were stage IA and IB. The descending use of therapies for stage I-III included steroids and diverse UV therapies followed by bexarotene, interferon-α, methotrexate, and extracorporal photophoresis. CONCLUSIONS: Diagnoses of cutaneous lymphomas belong to a vast spectrum of differential diagnoses. This registry describes frequent findings and shows rare variants. You can only diagnose what you know; accordingly, a collection of case reports, which we wish to encourage, can help in processing and specification of entities.


Assuntos
Linfoma Cutâneo de Células T/classificação , Linfoma Cutâneo de Células T/epidemiologia , Papulose Linfomatoide/epidemiologia , Micose Fungoide/epidemiologia , Neoplasias Cutâneas/classificação , Neoplasias Cutâneas/epidemiologia , Alemanha/epidemiologia , Humanos , Antígeno Ki-1 , Papulose Linfomatoide/patologia , Micose Fungoide/patologia
12.
Int J Dermatol ; 57(6): 675-680, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29603194

RESUMO

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a cutaneous T-cell lymphoma mainly affecting the hair follicle, which seems to represent a place of immune privilege phenomenon. OBJECTIVES: To explore a possible role of immune privilege (IP) in FMF analyzing the major histocompatibility complex (MHC) expression. METHODS: Immunohistochemistry for HLA-G and MHC-II was performed to formalin-fixed paraffin-embedded cutaneous skin biopsies of FMF patients (n = 43), conventional mycosis fungoides (CMF; n = 13), alopecia areata (AA; n = 13), and normal scalp skin (NS; n = 12). RESULTS: HLA-G expression was lower in FMF (34%: 14/41) and CMF (18%: 2/11) groups compared to alopecia areata (92%:11/12) and normal scalp skin group (100%: 12/12). MHC-II expression in hair follicle was greater in the FMF group (18/42: 43%) compared to AA (0%) and NS (0%). HLA-G and MHC-II expression in cellular infiltrate had no difference among FMF and CMF groups and was different compared to the AA group. CONCLUSIONS: Our data support the hypothesis of disruption of immune privilege based on the lower expression of HLA-G and higher expression of MHC-II in the follicular epithelium in mycosis fungoides compared to alopecia areata and normal scalp skin. The lack of difference between FMF and CMF groups did not support the role of these molecules as a driver of folliculotropism. The expression of MHC molecules seems to be different between neoplastic and inflammatory infiltrates. The definitive significance of expression of the MHC molecules remains unclear, and more studies are necessary to fully understand the role of these molecules in cutaneous lymphomas.


Assuntos
Linfoma Cutâneo de Células T/imunologia , Linfoma Cutâneo de Células T/patologia , Micose Fungoide/imunologia , Micose Fungoide/patologia , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/patologia , Fatores Etários , Idoso , Biópsia por Agulha , Brasil , Distribuição de Qui-Quadrado , Estudos de Coortes , Feminino , Antígenos HLA-G/imunologia , Folículo Piloso/patologia , Histocompatibilidade , Humanos , Imuno-Histoquímica , Incidência , Linfoma Cutâneo de Células T/epidemiologia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Estudos Prospectivos , Fatores Sexuais , Neoplasias Cutâneas/epidemiologia , Estatísticas não Paramétricas
13.
Int J Dermatol ; 57(3): 306-312, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29318586

RESUMO

BACKGROUND: Hypopigmented mycosis fungoides (hMF) is a rare subtype of mycosis fungoides. The aim of this study was to identify the clinical-epidemiological profile of our patient group and also to provide additional information about treatment responses and prognosis. METHODS: This is a cross-sectional retrospective observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS: Twenty patients with hMF were selected from a group of 102 patients diagnosed with MF. There was no gender difference (10 females and 10 males). Mean age at diagnosis was 43.85 years, and most patients had mixed or black skin color. The mean time between the onset of the lesions and the diagnosis was 66.75 months. Patients were equally distributed in stages IA (50%) and IB (50%). Photochemotherapy (psoralen and ultraviolet A) was the predominant therapeutic modality. The mean follow-up time was 7.25 years. In 10%, disease progression was observed. Death related to the disease occurred in one patient. CONCLUSIONS: The clinical and epidemiological profile of patients with hypopigmented MF found in our sample is in agreement with what is described in the literature, with the exception of the age at diagnosis, higher than expected. Diagnostic delay time, despite long, is also consistent with the medical literature; however, in this sample, we had two cases of disease progression, with death of one patient, despite the treatment, which is extremely important since hypopigmented MF is usually associated with good prognosis.


Assuntos
Hipopigmentação/diagnóstico , Hipopigmentação/tratamento farmacológico , Micose Fungoide/diagnóstico , Micose Fungoide/tratamento farmacológico , Adolescente , Adulto , Idade de Início , Idoso , Brasil/epidemiologia , Criança , Estudos Transversais , Diagnóstico Tardio , Progressão da Doença , Feminino , Seguimentos , Humanos , Hipopigmentação/complicações , Hipopigmentação/epidemiologia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/complicações , Micose Fungoide/epidemiologia , Terapia PUVA , Estudos Retrospectivos , Taxa de Sobrevida , Adulto Jovem
14.
J Am Acad Dermatol ; 78(6): 1077-1083.e4, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29203438

RESUMO

BACKGROUND: Mycosis fungoides (MF) and parapsoriasis are characterized by malignant proliferation and chronic inflammation, which may affect the risk for venous thromboembolism (VTE). OBJECTIVES: To examine the risk for VTE in patients with MF and parapsoriasis. METHODS: We conducted a nationwide population-based cohort study in Denmark to examine the relative risk (RR) of VTE in 525 patients with MF and 634 patients with parapsoriasis compared with that in sex- and age-matched controls from the general population. RESULTS: In patients with MF, the 10-year absolute risk for VTE was 3.4% (95% confidence interval [CI], 2.0-5.4). The adjusted RRs were 2.41 (95% CI, 1.49-3.90) for VTE and 4.01 (95% CI, 2.16-7.46) for pulmonary embolism. Notably, within the first 5 years after diagnosis with MF, the RR of pulmonary embolism was increased 6.7-fold (to 6.71 [95% CI, 2.86-15.72]). Patients with parapsoriasis had a 2.7-fold increased RR of VTE (to 2.67 [95% CI, 1.32-5.40]) in the absence of other established VTE risk factors. LIMITATIONS: We had no information regarding disease stage of MF and prescribed drugs. CONCLUSION: Patients with MF and parapsoriasis had an increased RR of VTE, although the absolute risk remained low. These findings should increase awareness of comorbidities in patients with MF and parapsoriasis.


Assuntos
Micose Fungoide/epidemiologia , Parapsoríase/epidemiologia , Sistema de Registros , Tromboembolia Venosa/epidemiologia , Adulto , Distribuição por Idade , Idoso , Estudos de Coortes , Comorbidade , Dinamarca/epidemiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Micose Fungoide/diagnóstico , Parapsoríase/diagnóstico , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Distribuição por Sexo , Tromboembolia Venosa/diagnóstico
15.
Eur J Dermatol ; 28(1): 44-49, 2018 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-29171395

RESUMO

BACKGROUND: Primary cutaneous lymphomas (PCLs) are a rare group of extranodal non-Hodgkin lymphomas, and epidemiological data in Mediterranean countries are scarce. OBJECTIVE: To investigate the incidence and characteristics of PCL in a single tertiary referral centre in Italy. MATERIALS & METHODS: A total of 141 PCL patients, seen over a 10-year follow-up period, were investigated. RESULTS: Incidence rate of PCL was 0.8 cases/100,000 person years. T-cell lymphoma represented 78.7% of all cases, the majority being early mycosis fungoides (MF) (64%; median age: 66 years), followed by lymphomatoid papulosis (LyP) (19%; median: age 48 years), and others (median age: 72 years), including eight cases of anaplastic large CD30+ T-cell lymphoma, four CD4+ small-medium pleomorphic T-cell lymphoproliferative disorder, four Sézary syndrome, one subcutaneous panniculitis-like T-cell lymphoma, one extranodal NK/T-cell lymphoma nasal-type, and one angioimmunoblastic T-cell lymphoma. B-cell lymphoma accounted for 21.3% of PCL, with 20 cases of cutaneous follicular centre B-cell (median age: 63 years), four primary cutaneous marginal zone, three primary cutaneous diffuse large B-cell, and three leg-type lymphoma. Complete remission within the first year after diagnosis occurred in 70.4% of MF, 61.9% of LyP, 78.9% of other T-cell lymphoma, and 93.1% of B-cell lymphoma cases. Based on a Cox proportional hazard regression model, age, gender, stage, and lactate dehydrogenase and ß2-microglobulin blood levels did not predict clinical remission of MF or LyP. CONCLUSIONS: The incidence and characteristics of PCL in Italy are similar to those in other European countries. PCLs may be diagnosed at very early stages with good prognosis.


Assuntos
Linfoma Cutâneo de Células T/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Incidência , Itália/epidemiologia , Estimativa de Kaplan-Meier , Linfoma de Células B/epidemiologia , Linfoma Cutâneo de Células T/imunologia , Linfoma Cutâneo de Células T/patologia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Micose Fungoide/imunologia , Micose Fungoide/patologia , Prognóstico , Estudos Retrospectivos , Distribuição por Sexo , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/patologia , Fatores de Tempo
17.
Actas dermo-sifiliogr. (Ed. impr.) ; 108(6): 564-570, jul.-ago. 2017. tab, ilus
Artigo em Espanhol | IBECS | ID: ibc-164511

RESUMO

La micosis fungoide (MF) es el linfoma cutáneo primario de células T más frecuente. Su aparición en la infancia es excepcional. Objetivos: Describir las características epidemiológicas, clínicas, histopatológicas e inmunofenotípicas de los pacientes con MF. Describir los tratamientos utilizados y la evolución. Material y método: Se incluyeron todos los pacientes admitidos en el Hospital de Pediatría Dr. J. P. Garrahan (Argentina) en el período comprendido entre agosto de 1988 y julio de 2014 con diagnóstico clínico e histopatológico de MF. Resultados: Se diagnosticaron 14 pacientes con MF. La distribución por sexo fue M/F: 1:1,33. La edad media al diagnóstico fue de 11,23 años (rango: 8 a 15 años). El tiempo promedio de evolución hasta el momento del diagnóstico fue de 3 años y 6 meses (rango: 4 meses a 7 años). Todos los pacientes presentaron la forma clínica hipopigmentada y en el 42% se asoció la forma clásica. El 50% (n = 7) exhibió un inmunofenotipo CD8 positivo de forma exclusiva. El 78% presentó estadio IB. La fototerapia fue el tratamiento de elección. Cuatro pacientes tuvieron por lo menos una recaída y 3 demostraron progresión de su enfermedad a nivel cutáneo. La evolución fue favorable en todos los casos. Conclusiones: La MF es una entidad infrecuente en la infancia, siendo la forma hipopigmentada la más frecuente. Su diagnóstico es tardío debido a la similitud con otras enfermedades hipopigmentadas frecuentes en la niñez. A pesar de tener un buen pronóstico, presenta alta tasa de recidivas y requiere un seguimiento a largo plazo (AU)


Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. Objectives: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. Material and method: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. Results: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. Conclusions: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary (AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Micose Fungoide/epidemiologia , Linfoma Cutâneo de Células T/epidemiologia , Neoplasias Cutâneas/patologia , Distribuição por Idade e Sexo , Micose Fungoide/patologia , Linfoma Cutâneo de Células T/patologia , Progressão da Doença , Estudos Retrospectivos , Pitiríase Liquenoide/patologia
18.
J Cutan Med Surg ; 21(6): 507-512, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28614957

RESUMO

BACKGROUND: Lymphomatoid papulosis is one of the primary cutaneous CD30+ T-cell lymphoproliferative disorders. Although considered a benign disease, lymphomatoid papulosis has been associated potentially with an increased risk of secondary hematolymphoid malignancies. OBJECTIVE: The aim of this study was to assess the clinical characteristics and histologic subtypes of lymphomatoid papulosis, identify the prevalence and types of secondary hematolymphoid malignancies, and determine the potential risk factors for development of these hematolymphoid malignancies. METHODS AND MATERIALS: A retrospective chart review was performed for all histologically confirmed cases of lymphomatoid papulosis between 1991 and 2016. RESULTS: Seventy patients with lymphomatoid papulosis were identified. Thirty patients (43%) experienced a secondary hematolymphoid malignancy. Twenty-four (80%) of the hematolymphoid malignancies occurred after the onset of lymphomatoid papulosis. Older age at diagnosis of lymphomatoid papulosis, male sex, histology type B, and the presence of T-cell receptor gene rearrangement are associated with higher risk of developing hematolymphoid malignancy. CONCLUSION: Lymphomatoid papulosis is associated with increased risk of developing secondary hematolymphoid malignancies, particularly mycosis fungoides and cutaneous anaplastic large cell lymphoma.


Assuntos
Leucemia Linfocítica Crônica de Células B/epidemiologia , Linfoma Cutâneo de Células T/epidemiologia , Papulose Linfomatoide/tratamento farmacológico , Papulose Linfomatoide/epidemiologia , Micose Fungoide/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adolescente , Adulto , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Canadá/epidemiologia , Feminino , Rearranjo Gênico , Genes Codificadores dos Receptores de Linfócitos T/genética , Doença de Hodgkin/epidemiologia , Humanos , Linfoma Anaplásico de Células Grandes/epidemiologia , Papulose Linfomatoide/diagnóstico , Papulose Linfomatoide/patologia , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Taxa de Sobrevida , Adulto Jovem
19.
Clin Lymphoma Myeloma Leuk ; 17(8): 520-526.e2, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-28655598

RESUMO

BACKGROUND: Management of mycosis fungoides and Sézary syndrome (MF/SS) is complex, and randomized evidence to guide treatment is lacking. The institutional treatment volumes for MF/SS might vary widely nationally and influence patient survival. PATIENTS AND METHODS: Using the National Cancer Database, we identified patients with a diagnosis of MF/SS from 2004 to 2011 in the United States who had received treatment at a reporting facility. The patients were grouped into quintiles according to their treatment facility's average annual treatment volume (ATV). The characteristics associated with ATV were identified and compared using χ2 tests. Overall survival (OS) was compared among the ATV quintiles using the Kaplan-Meier method with log-rank tests and multivariable Cox regression with hazard ratios (HRs). OS was also analyzed using the annual patient volume as a continuous variable. RESULTS: A total of 2205 patients treated at 374 facilities were included for analysis. The ATV quintile cutoffs were 1, 3, 6, and 9 patients. With a median follow-up period of 59 months, the 5-year estimated OS survival increased with ATV from 56.7% in the lowest quintile (≤ 1 patient annually) to 83.8% in the highest quintile (> 9 patients annually; P < .001). On multivariable analysis, greater ATV was associated with improved survival when analyzed as a continuous variable (HR, 0.96 per patient per year; 95% confidence interval, 0.94-0.98; P < .001) and when comparing the highest quintile to the lowest quintile (HR, 0.46; 95% confidence interval, 0.39-0.55). CONCLUSION: The present national database analysis demonstrated that higher facility ATV is associated with improved OS for patients with MF/SS. Further study is needed to determine the underlying reasons for improved survival with higher facility ATV.


Assuntos
Institutos de Câncer , Pacientes Internados , Micose Fungoide/epidemiologia , Síndrome de Sézary/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/diagnóstico , Micose Fungoide/mortalidade , Micose Fungoide/terapia , Estadiamento de Neoplasias , Vigilância em Saúde Pública , Síndrome de Sézary/diagnóstico , Síndrome de Sézary/mortalidade , Síndrome de Sézary/terapia , Fatores Socioeconômicos , Taxa de Sobrevida
20.
Arch Dermatol Res ; 309(6): 453-459, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28516243

RESUMO

Folliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides (MF) where atypical T-cells invade the hair follicles. The objective was to assess the clinical features, risk factors for progression, long-term outcome and response to treatment modalities in a large cohort of FMF patients. We, therefore, conducted a single-center retrospective study, reviewing 114 patients with FMF seen from 1987 to 2015 at the cutaneous T-cell lymphoma clinic of the MD Anderson Cancer Center. The mean age at diagnosis of FMF was 57.1 ± 13.5 years. The male to female ratio was 1.2:1. MF stage IA (n = 50, 43.9%) was the most common diagnosed stage, followed equally by stages IB and IIB (n = 23, 20.2%, respectively). Eighty-six patients (75.4%) suffered from pruritus. Concomitant hair loss was observed in 37 (32.5%) subjects. The median number of different treatment modalities used per patient was 4 (range 1-12). Low-dose radiation was beneficial in clearing therapy refractory lesions. The overall survival was influenced by several factors, including advanced age (>65), late stages and the presence of large cell transformation (LCT). Thirty-three of 113 patients (29.2%) progressed to more advanced stages and 26 (23.0%) died, with the cause of death being MF in 11 (9.7%) subjects. In conclusion, patients with FMF have a worse overall 10-year survival rate compared with other MF variants. Several factors, including stage, advanced age and the presence of LCT impact survival. Multiple different treatment approaches may be needed to achieve a good clinical response and to prevent disease progression.


Assuntos
Folículo Piloso/patologia , Micose Fungoide/epidemiologia , Micose Fungoide/patologia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/patologia , Adulto , Idoso , Alopecia/epidemiologia , Alopecia/patologia , Biópsia , Terapia Combinada , Progressão da Doença , Epiderme/patologia , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Micose Fungoide/terapia , Estadiamento de Neoplasias , Cuidados Paliativos/métodos , Prurido , Dosagem Radioterapêutica , Estudos Retrospectivos , Fatores de Risco , Neoplasias Cutâneas/terapia , Taxa de Sobrevida , Resultado do Tratamento , Irradiação Corporal Total
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