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1.
Br J Dermatol ; 191(2): 233-242, 2024 Jul 16.
Artículo en Inglés | MEDLINE | ID: mdl-38595050

RESUMEN

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population are scarce. OBJECTIVES: To characterize the epidemiological, clinical, histopathological and prognostic features of paediatric LyP. METHODS: This was a retrospective multicentre international cohort study that included 87 children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years at disease onset were included. LyP diagnosis was made in each centre, based on clinicopathological correlation. RESULTS: Eighty-seven patients from 12 centres were included. Mean age at disease onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2 : 1. Mean time between the onset of the first cutaneous lesions and diagnosis was 1.3 years (range 0-14). Initial misdiagnosis concerned 26% of patients. LyP was most often misdiagnosed as pityriasis lichenoides et varioliformis acuta, insect bites or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned in 21% of patients. The main histological subtype was type A in 55% of cases. When analysed, monoclonal T-cell receptor rearrangement was found in 77% of skin biopsies. The overall survival rate was 100%, with follow-up at 5 years available for 33 patients and at 15 years for 8 patients. Associated haematological malignancy (HM) occurred in 10% of cases (n = 7/73), including four patients with mycosis fungoides, one with primary cutaneous anaplastic large cell lymphoma (ALCL), one with systemic ALCL and one with acute myeloid leukaemia. If we compared incidence rates of cancer with the world population aged 0-19 years from 2001 to 2010, we estimated a significantly higher risk of associated malignancy in general, occurring before the age of 19 years (incidence rate ratio 87.49, 95% confidence interval 86.01-88.99). CONCLUSIONS: We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall, the disease prognosis is good, with excellent survival rates for all patients. Owing to an increased risk of associated HM, long-term follow-up should be recommended for patients with LyP.


Lymphomatoid papulosis is a very rare skin condition caused by an abnormal increase in white blood cells (called 'lymphocytes') in the skin. The condition rarely affects children, so most of the scientific data published about this disease focuses on adults. This study involved 12 academic dermatology centres in Europe, the Middle East and North America, and gathered data from about 87 children who presented with symptoms of lymphomatoid papulosis before the age of 19 years. The aim of this study was to better describe this disease in the paediatric population and discuss its treatment options and evolution. We found that the presentation of the disease in children is roughly the same as in adults. Safe and effective treatment options exist. The disease is not life threatening, but it requires investigation by a dermatologist, both to make a careful diagnosis and to monitor it as sometimes associated cancers that originate from blood cells can occur, mostly on the skin.


Asunto(s)
Papulosis Linfomatoide , Neoplasias Cutáneas , Humanos , Papulosis Linfomatoide/patología , Papulosis Linfomatoide/epidemiología , Masculino , Estudios Retrospectivos , Niño , Femenino , Adolescente , Preescolar , Lactante , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/mortalidad , Edad de Inicio , Pronóstico , Errores Diagnósticos/estadística & datos numéricos , Pitiriasis Liquenoide/epidemiología , Pitiriasis Liquenoide/patología , Pitiriasis Liquenoide/diagnóstico , Mordeduras y Picaduras de Insectos/epidemiología , Mordeduras y Picaduras de Insectos/complicaciones , Molusco Contagioso/epidemiología , Molusco Contagioso/patología , Molusco Contagioso/diagnóstico
2.
Acta Derm Venereol ; 103: adv10306, 2023 Oct 30.
Artículo en Inglés | MEDLINE | ID: mdl-37902466

RESUMEN

Mycosis fungoides and Sézary syndrome are rare and largely incurable types of cutaneous T-cell lymphoma with limited therapeutic options. In 1984 Bunn et al. reported that interferon alpha is an efficient monotherapy in cutaneous T-cell lymphoma and 14 years later it was shown in a prospective, randomized trial that a combination of interferon alpha and psoralen plus ultraviolet A therapy (PUVA) is most efficient in the treatment of cutaneous T-cell lymphoma. Since then interferon alpha as single agent or, most often, in combination with phototherapy and/or retinoids has been integrated as standard of care in cutaneous T-cell lymphoma guidelines worldwide. However, production of interferon alpha was discontinued recently worldwide and pegylated interferon alpha-2a (PEG-IFNα) has been used as an alternative therapy. In contrast to numerous interferon alpha studies, only a few studies focusing on PEG-IFNα are available. Therefore, the aim of this study was to conduct a retrospective data collection to report on the efficacy, adverse events and therapy regimens of PEG-IFNα in cutaneous T-cell lymphoma. In 28 patients with cutaneous T-cell lymphoma treated in Germany and in the Netherlands, 36% of patients achieved complete remission, 36% partial remission and 29% stable disease. Eighteen percent of patients developed adverse events during therapy, which led to the discontinuation of PEG-IFNα therapy in 2 patients. The most common concomittant therapies were oral PUVA phototherapy and local radiotherapy. In conclusion, PEG-IFNα, especially in combination with skin-directed therapies, is an effective treatment option for cutaneous T-cell lymphoma in clinical practice.


Asunto(s)
Linfoma Cutáneo de Células T , Neoplasias Cutáneas , Humanos , Interferón-alfa/efectos adversos , Linfoma Cutáneo de Células T/tratamiento farmacológico , Polietilenglicoles/efectos adversos , Estudios Retrospectivos , Neoplasias Cutáneas/tratamiento farmacológico
3.
J Dtsch Dermatol Ges ; 21(9): 992-1002, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37401138

RESUMEN

BACKGROUND AND OBJECTIVES: The effect of mogamulizumab in cutaneous T-cell lymphoma (CTCL) on T cells (TC) in the peripheral blood and its potential role to navigate treatment intervals are explored. METHODS: We investigated within a retrospective monocentric analysis the effect of mogamulizumab on the CD3+ TC and the aberrant T cell population (TCP), i.e., the CD4+ /CD7- and the CD4+ /CD26- TC, analyzed by flow cytometry. RESULTS: Thirteen patients with CTCL were included. After four cycles there was a mean reduction of 57% in CD3+ TC, 72% in the CD4+ /CD7- and 75% in the CD4+ /CD26- TCP compared to the individual baseline of each patient. The reduction in CD4+ /CD7+ and CD4+ /CD26+ TC was lower, averaging 54% and 41%. A significant decrease in aberrant TCP was already evident after the first administration. A median plateau of TCP already occurred during the IP. Progressive disease occurred in 5/13 patients without a clear correlation to aberrant TCP. CONCLUSIONS: Already after one dose of mogamulizumab, aberrant TCP and, to a lesser extent, normal TC decrease. We did not observe a clear correlation between TCP and the efficacy of mogamulizumab, but further studies with larger numbers of patients are needed.


Asunto(s)
Linfoma Cutáneo de Células T , Micosis Fungoide , Síndrome de Sézary , Neoplasias Cutáneas , Humanos , Linfocitos T/metabolismo , Dipeptidil Peptidasa 4/análisis , Dipeptidil Peptidasa 4/metabolismo , Dipeptidil Peptidasa 4/uso terapéutico , Micosis Fungoide/patología , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patología , Estudios Retrospectivos
10.
Nuklearmedizin ; 59(3): 260-268, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32403138

RESUMEN

INTRODUCTION: While surgical treatment is preferred for Graves' disease with active forms of GO, there are various concepts for treating inactive forms of GO. The goal of radioiodine therapy is to resolve immunogenic hyperthyroidism by damaging the thyroid cells.The effects of the radioiodine dose on an associated inactive GO remain unclear, however. METHODOLOGY: We conducted a retrospective analysis of 536 patients who received first-time radioiodine therapy to treat Graves' hyperthyroidism. Patients without GO always received 200 Gy of iodine-131. Before the introduction of a differentiated treatment concept, patients with GO also received 200 Gy, while afterwards they received 300 Gy. For further analysis, we formed three patient groups based on GO diagnosis and administered radiation dose and compared their results. The main research question focused on the effect of an increased dose on Graves' orbitopathy. The sub-questions addressed the resolution rate achieved with the higher dose as well as the development of GO in patients who received radioiodine therapy. RESULTS: The results show that GO symptoms were improved after radioiodine treatment in 68.5 % of patients treated with 300 Gy but only in 47.5 % of the patients treated with 200 Gy (p = 0.003). While in the 300 Gy group, hyperthyroidism was resolved in 93.2 % of patients, this was achieved in only 68.8 % of patients in the 200 Gy group (p 

Asunto(s)
Oftalmopatía de Graves/radioterapia , Radioisótopos de Yodo/uso terapéutico , Dosis de Radiación , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Dosificación Radioterapéutica , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven
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