يعرض 1 - 10 نتائج من 282 نتيجة بحث عن '"Ram‐Wolff, Caroline"', وقت الاستعلام: 1.22s تنقيح النتائج
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    دورية أكاديمية

    المساهمون: Institut National de la Santé et de la Recherche Médicale, Société Française de Dermatologie et de Pathologie Sexuellement Transmissible

    المصدر: Skin Health and Disease ; volume 4, issue 2 ; ISSN 2690-442X 2690-442X

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    دورية أكاديمية

    المصدر: JEADV Clinical Practice ; volume 3, issue 2, page 668-671 ; ISSN 2768-6566 2768-6566

    الوصف: Cutaneous T‐cell lymphomas (CTCLs) are unique non‐Hodgkin lymphomas due to the proliferation of skin‐homing T cells. Sezary syndrome (SS), a subtype of CTCL, typically presents as erythroderma with specific blood involvement and seldomly evolves from tumor‐stage mycosis fungoides (MF). The article examines the case of a 62‐year‐old female patient who developed SS while under atezolizumab treatment for tumor‐stage transformed MF. Postdiagnosis, she underwent treatments including topical corticosteroids, phototherapy, and other drug regimens, experiencing both complete responses and relapses over the years. Three years postdiagnosis, her condition deteriorated with facial tumor lesions, confirmed as MF. Despite multiple treatments, including oral drugs, radiation therapy, and a range of other therapeutic agents, her condition did not show sustained improvement. The patient was then administered atezolizumab, an antibody targeting programmed death ligand 1 (PD‐L1). Although initial results saw a disappearance of her skin tumors, the subsequent cycles led to symptoms consistent with stage IVA1 SS, coupled with an evident shift in malignant T‐cell clone dominance. Atezolizumab, a humanized monoclonal antibody, impedes the interaction of PD‐L1 with its receptor PD1, which is notably overexpressed in malignant T‐cells of SS. This case illustrates the emergence of SS during the course of atezolizumab treatment for a transformed MF condition, underlining the potential risks and complexities in using PD‐L1 inhibitors for treating CTCL.

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    دورية أكاديمية
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    دورية أكاديمية

    المساهمون: UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (SLuc) Service d'anatomie pathologique, UCL - (SLuc) Service de chirurgie plastique, UCL - (SLuc) Centre de l'allergie, UCL - (SLuc) Centre de malformations vasculaires congénitales

    المصدر: Dermatology, Vol. 239, no.1, p. 132-139 (2023)

    الوصف: BACKGROUND: Our objective was to describe the clinical, histological characteristics, and disease outcome of a cohort of mycosis fungoides (MF) diagnosed during childhood including disease status at adulthood. METHODS: This is a retrospective multicentre survey of patients aged under 18 years at diagnosis with histologically confirmed MF. Patients' clinical and histological characteristics, treatments, and disease outcome (for patients followed for more than 12 months) were analysed. RESULTS: Forty-six patients were included (median age at diagnosis: 11 years; M:F sex ratio: 3:1) with 39 (85%) followed for at least 12 months. Thirty-nine patients (85%) had stage I MF. Hypopigmented patches were observed in 48% and folliculotropism in 43% patients. Immunophenotype of the skin infiltrate was predominantly CD8+ in 17% of patients. Initial management included a wait-and-see strategy in 6/39 (15%), skin-directed treatment in 27 (69%), and systemic treatment in 6 (15%) patients, respectively, with partial or complete clinical response (PR or CR) observed in 28 patients (72%). 14/39 patients (36%) relapsed after initial response. After a median follow-up period of 54 months, disease status at last news was PR or CR in 31/39 (79%), stable disease in 6 (15%), and progression in 2 (5%) patients. Histological transformation was observed in 3/39 (8%). Of the 15 patients followed until adulthood, 13 (87%) had persistent MF. DISCUSSION: This survey confirms the high frequency of hypopigmented and folliculotropic lesions and of CD8+ immunophenotype compared to adult MF patients. The long-term course is usually indolent but transformation may occur sometimes long after disease onset and the disease may persist during adulthood.

    العلاقة: boreal:277393; http://hdl.handle.net/2078.1/277393Test; info:pmid/36349768; urn:ISSN:1018-8665; urn:EISSN:1421-9832

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    دورية أكاديمية

    المصدر: JEADV Clinical Practice ; volume 2, issue 4, page 727-738 ; ISSN 2768-6566 2768-6566

    الوصف: Background Angioimmunoblastic T‐cell lymphoma (AITL) is one of the most frequent peripheral T‐cell lymphomas (PTCL) in western countries. Skin involvement is common and may reveal the malignancy. Despite its frequency, skin involvement in AITL has been poorly described. Objectives We aimed to analyze the cutaneous expression of PTCL of TFH origin and its prognostic impact. Methods We conducted a multicenter retrospective cohort study by retrieving histopathological reports including the mention ‘AITL’ or ‘PTCL with T‐follicular helper phenotype’ (PTCL‐TFH) from five French tertiary hospital centers. Results From 2000 to 2022, we reviewed 382 histopathological records and identified 52 AITL cases and 5 PTCL‐TFH cases with cutaneous involvement. Thirty‐two (56%) patients were males with a mean age of 63 years. Fifty‐six (98%) patients presented with lymphadenopathy, 32 (56%) splenomegaly and 17 (30%) hepatomegaly. B signs were present in 34 (60%) patients. Skin lesions were present on the lower limbs in 44 (77%) patients, trunk in 38 (67%) patients, upper limbs in 35 (61%) and head in 27 (47%). Macules and papules were the most frequent lesions found in 47 (82%) patients, followed by nodules in 10 (17%) patients, erythemato‐squamous plaques in 10 (17%) patients, purpura in 9 (16%), urticaria in 9 (16%) and blisters in 5 (9%) patients. Erythroderma affected seven patients (12%). A skin biopsy was taken in 50 patients and revealed a specific lymphomatous infiltrate in 36 cases. A dominant skin T‐cell clone was detected in 13 out of 17 (76%) patients. Among the 14 patients with a nonspecific dermatitis, various histopathological patterns were observed including interface dermatitis, psoriasiform dermatitis, vasculitis, bullous dermatitis, granulomatous dermatitis and thrombotic vasculopathy. After a median follow‐up of 24 months (range, 0–121 months), median overall survival was 121 months (95% CI, 25.2–NA). At last follow‐up, 33 patients (58%) were alive, 20 (35%) were in complete remission and 7 (12%) were ...

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    دورية أكاديمية

    المصدر: JEADV Clinical Practice ; volume 2, issue 3, page 625-628 ; ISSN 2768-6566 2768-6566

    الوصف: Primary cutaneous follicle centre lymphomas (PCFCLs) are cutaneous proliferation of centrocytes and centroblasts, with a generally indolent evolution. Secondary systemic spread of disease is a rare event, which significantly impairs prognosis. Recently published study by Zhou and al. proposed criteria aiming to predict such evolution at initial stage: (i) rearrangement of Bcl2 , (ii) Ki67% index <30% and (iii) 2 mutations among the CREBBP, KMT2D, EZH2 and EP300.1 genes. We herein report two cases of PCFCLs with systemic evolution. Both patients had been initially diagnosed with localised skin disease, and developed, several years after, a secondary systemic involvement. Skin biopsies at the initial stage and systemic spread were analysed by histology, immunohistochemistry, fluorescence in situ hybridisation, and by DNA sequencing of a panel of 54 genes frequently mutated in lymphomas. Both patients showed no rearrangement of Bcl2 , >30% Ki67% indexes and no mutations among the four proposed genes. These findings run contrary to these previously proposed criteria, highlighting the molecular heterogeneity of this rare disease.

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    دورية أكاديمية
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    دورية أكاديمية

    المصدر: The Lancet ; volume 401, issue 10392, page 1941-1950 ; ISSN 0140-6736

    مصطلحات موضوعية: General Medicine

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    دورية أكاديمية

    المساهمون: Hôpital Henri Mondor, Epidemiology in Dermatology and Evaluation in Therapeutics (EpiDermE), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Hopital Saint-Louis AP-HP (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris Cité (UPCité), BoRdeaux Institute in onCology (Inserm U1312 - BRIC), Université de Bordeaux (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux, CHU Montpellier, Centre Hospitalier Régional Universitaire Montpellier (CHRU Montpellier), Immunology and New Concepts in ImmunoTherapy (INCIT), Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre Hospitalier Universitaire de Nantes = Nantes University Hospital (CHU Nantes)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), Centre Hospitalier Universitaire de Nantes = Nantes University Hospital (CHU Nantes), Centre d’Investigation Clinique de Nantes (CIC Nantes), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Universitaire de Nantes = Nantes University Hospital (CHU Nantes), Centre Hospitalier Régional Universitaire CHU Lille (CHRU Lille), Centre Hospitalier Universitaire Brugmann Bruxelles (CHU), Institut Jules Bordet Bruxelles, Faculté de Médecine Bruxelles (ULB), Université libre de Bruxelles (ULB)-Université libre de Bruxelles (ULB), CHU Rouen, Normandie Université (NU), Université de Lausanne = University of Lausanne (UNIL), Centre Hospitalier Universitaire Vaudois = Lausanne University Hospital Lausanne (CHUV), Hôpital Cochin AP-HP, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Universitaire de Rennes CHU Rennes = Rennes University Hospital Ponchaillou, Geneva University Hospitals and Geneva University, Hôpital Nord CHU - APHM, Centre hospitalier de Valence, Centre Hospitalier Universitaire de Nice (CHU Nice)

    المصدر: ISSN: 0007-0963.

    مصطلحات موضوعية: [SDV]Life Sciences [q-bio]

    الوصف: International audience ; The results of this study suggest that a progressive discontinuation of mogamulizumab should be considered once response is obtained in Sezary syndrome patients, even after a treatment of short-duration. This may possibly minimize the risk of adverse effects, costs, and resistance to mogamulizumab. Additionally, our study emphasizes that mogamulizumab rechallenge should be encouraged as first-choice for relapsing patients. ; No abstract available

    العلاقة: info:eu-repo/semantics/altIdentifier/pmid/38660811; inserm-04559196; https://inserm.hal.science/inserm-04559196Test; PUBMED: 38660811

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    دورية أكاديمية