Publications by authors named "Nicolay J"

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of the heterogeneous group of cutaneous T-cell lymphomas (CTCL). With the expansion of the biologic treatment landscape, new treatment options have become available in recent years, most notably the C-C chemokine receptor 4 (CCR4)-directed monoclonal antibody mogamulizumab. Based on the phase III pivotal trial, mogamulizumab is recommended by the German S2k guidelines for the second-line treatment of stage IB and above SS and MF, after at least one prior systemic therapy.

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Primary cutaneous T cell lymphomas (CTCL) are characterized by high relapse rates to initially highly effective therapies. Combination therapies have proven beneficial, particularly if they incorporate extracorporeal photopheresis (ECP). The NF-κB inhibitor dimethyl fumarate (DMF) has proven a new, effective drug in CTCL in a clinical phase II study.

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Purpose: We aim to determine the current treatment patterns and recommendations among physicians for cutaneous lymphomas and to identify the types of skin lymphomas for which existing radiation regimens need improvement.

Methods And Materials: A questionnaire from the European Organisation for Research and Treatment of Cancer was distributed to all members of the Cutaneous Lymphoma Tumour Group and Radiation Oncology Scientific Council. This online survey included 13 questions regarding treatment practices for patients with cutaneous lymphoma.

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Cutaneous T-cell lymphomas (CTCLs) are a heterogeneous group of diseases characterised by abnormal neoplastic T-cell growth in the skin. Mycosis fungoides (MF), the most common CTCL, manifests as erythematous skin patches and/or plaques, tumours or erythroderma. The disease may involve blood, lymph nodes and rarely viscera.

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Article Synopsis
  • * Radiation oncologists must make tailored decisions for patient treatment, but these choices would benefit from more clinical evidence and research.
  • * The article calls for a prospective trial to better understand how different radiation doses affect patient outcomes in primary cutaneous lymphoma treatment.
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  • Interferon-alpha is crucial for treating cutaneous T-cell lymphomas (CTCL), but the approved version (IFN-α2a) has been unavailable since January 2020, prompting the use of pegylated interferon-α2a (pegIFN-α2a), which is not officially approved for this condition.
  • A study involving 70 CTCL patients from twelve German skin centers found a 55.2% overall response rate to pegIFN-α2a, with common adverse effects leading to a 50% discontinuation rate within about 63 weeks.
  • The findings suggest that pegIFN-α2a therapy may have similar efficacy and side effects as the discontinued IF
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The diagnosis of Sézary syndrome (SS) relies on the identification of blood Sézary cells (SC) by different markers via flow cytometry. Treatment of SS is challenging since its pathogenesis is characterized by cell death resistance rather than hyperproliferation. In this study, we establish an integrated approach that considers both the expression of SC markers and sensitivity to cell death both spontaneously and upon in vitro treatment.

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Article Synopsis
  • * Primary cutaneous B-cell lymphomas have undergone a significant review, leading to the establishment of new classifications, including a distinct category for cutaneous follicle center lymphomas.
  • * The document emphasizes the role of genomic and molecular data in improving classifications, although further research is needed to fully characterize these factors in primary cutaneous lymphomas.
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Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) is a subtype of cutaneous B-cell lymphoma with unfavorable prognosis usually requiring aggressive polychemotherapy for disease control. Only single cases of spontaneous regression of PCDLBCL, LT are reported in the literature, peaking 3 months post-biopsy following a clinical history of no longer than 1 year. Here, we report the first case of a spontaneously relapsing and remitting PCDLBCL, LT with complete regression after a clinical history of more than 9 years and thus an atypically indolent clinical course.

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Article Synopsis
  • Mycosis fungoides (MF) and Sézary syndrome (SS) are chronic skin cancers that require various treatment strategies; interferon-α (IFN-α) has been used for years, but only the pegylated form, PEG-IFN-α-2a, remains available, though not officially approved for these conditions.
  • A study involving 105 patients across 10 countries assessed the effectiveness and safety of PEG-IFN-α-2a, focusing on the time to next treatment (TTNT) as a measure of its clinical benefit.
  • Results showed a 53.3% overall response rate with better TTNT for patients receiving combination therapy (10.4 months) compared
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  • Lymphomatoid papulosis (LyP) is a rare skin disorder affecting T-cells, and there's limited information about its impact on children.
  • A study analyzed data from 87 pediatric patients diagnosed with LyP over a 24-year period, noting that most cases were initially misdiagnosed and the average age at onset was 7 years.
  • The prognosis appears positive, with a 100% overall survival rate, although 10% of patients had associated blood cancers, indicating a significantly higher risk of malignancy compared to the general youth population.
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Background: Primary cutaneous B-cell lymphomas (CBCL) are a group of rare malignant skin diseases that represent approximately 20%-30% of all primary cutaneous lymphomas (PCL). Previous studies revealed impaired health-related quality of life (HRQoL) in patients diagnosed with primary cutaneous T-cell lymphoma (CTCL). Currently, only small-sized studies investigated HRQoL in CBCL patients and lacked detailed analysis of respective subtypes.

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Background: Primary cutaneous T-cell lymphomas (CTCLs) are rare lymphoproliferative malignancies characterized by significant morbidity and mortality in advanced disease stages. As curative approaches apart from allogeneic stem cell transplantation are lacking, establishing new treatment options, especially combination therapies, is crucial.

Methods: This retrospective study included 11 patients with SS or MF receiving therapy with mogamulizumab in combination with ECP from four European expert centers.

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On behalf of the EORTC Cutaneous Lymphoma Tumours Group (EORTC-CLTG) and following up on earlier versions published in 2006 and 2017 this document provides an updated standard for the treatment of mycosis fungoides and Sézary syndrome (MF/SS). It considers recent relevant publications and treatment options introduced into clinical practice after 2017. Consensus was established among the authors through a series of consecutive consultations in writing and a round of discussion.

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  • The study introduces HEPNET, a deep learning model designed to accurately distinguish between intrahepatic cholangiocarcinoma and colorectal liver metastasis using H&E-stained whole-slide images.
  • HEPNET was trained on a large dataset from 456 patients and demonstrated high accuracy, achieving 96.5% on an internal test set and 98.1% on an external validation set, outperforming experienced pathology experts.
  • The model aims to improve routine pathology practices by providing a reliable diagnostic tool, potentially streamlining the diagnostic process in pathology laboratories.
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Targeted therapies for cutaneous T-cell lymphoma (CTCL) are limited and curative approaches are lacking. Furthermore, relapses and drug induced side effects are major challenges in the therapeutic management of patients with CTCL, creating an urgent need for new and effective therapies. Pathologic constitutive NF-κB activity leads to apoptosis resistance in CTCL cells and, thus, represents a promising therapeutic target in CTCL.

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Background: Consensus about the definition and classification of 'plaque' in mycosis fungoides is lacking.

Objectives: To delineate a comprehensive view on how the 'plaque' entity is defined and managed in clinical practice; to evaluate whether the current positioning of plaques in the TNMB classification is adequate.

Methods: A 12-item survey was circulated within a selected panel of 22 experts (pathologists, dermatologists, haematologists and oncologists), members of the EORTC and International Society for Cutaneous Lymphoma.

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Leukemic cutaneous T-cell lymphomas (L-CTCL) are lymphoproliferative disorders of skin-homing mature T-cells causing severe symptoms and high mortality through chronic inflammation, tissue destruction, and serious infections. Despite numerous genomic sequencing efforts, recurrent driver mutations have not been identified, but chromosomal losses and gains are frequent and dominant. We integrated genomic landscape analyses with innovative pharmacologic interference studies to identify key vulnerable nodes in L-CTCL.

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Hintergrund: Chlormethin-Gel ist in Europa zur Therapie von Patienten mit Mycosis fungoides in allen Krankheitsstadien zugelassen. Die optimalen Behandlungsregime hinsichtlich Frequenz, Dosierung, Kombinations- oder Erhaltungstherapien sind noch nicht vollständig etabliert.

Methodik: Zehn in der Erforschung und Behandlung kutaner T-Zell-Lymphome erfahrene Experten aus Deutschland, Österreich und der Schweiz (DACH-Region) wurden schriftlich zu Indikation, Anwendungsfrequenz, Beurteilung des Therapieerfolgs, Begleittherapie, Nebenwirkungen, Kombinationstherapien in späteren Krankheitsstadien, Erhaltungstherapie und Adhärenz im Rahmen der Therapie der Mycosis fungoides mit Chlormethin-Gel befragt.

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