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An Update on EBV-related Cutaneous Lymphoproliferative Disorders: a Systematic Review.

Actas Dermosifiliogr

November 2024

Universidad de Zaragoza, Zaragoza, España; Servicio de Anatomía Patológica, Hospital Clínico Universitario Lozano Blesa, Zaragoza, España.

Epstein Barr virus (EBV) positive B lymphoproliferative disorders (LPD) with cutaneous involvement include a series of rare entities that go from indolent processes to aggressive lymphomas. B-cell EBV+ LPD mainly affect immunocompromised patients while T-cell EBV+ LPD are more prevalent in specific geographic regions such as Asia, Central America, and South America. Since the latest WHO-EORTC classification of cutaneous lymphomas in 2018, significant changes have been included in the new classifications of hematological malignancies.

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Epstein-Barr virus-positive, primary cutaneous marginal zone lymphoma, with transformation: Case report and review of the literature.

Am J Clin Pathol

September 2024

Department of Pathology, Division of Dermatology, Department of Hematology and Hematopoietic Cell Transplantation, and Beckman Research Institute, City of Hope Medical Center, Duarte, CA, US.

Article Synopsis
  • Epstein-Barr Virus (EBV) positive primary cutaneous marginal zone lymphoma (PCMZL) is rare and can transform into more aggressive forms, like plasmablastic lymphoma (PBL), which is often fatal.
  • The case discussed involves a patient with multifocal PCMZL who experienced a sequence of transformations, eventually leading to disease progression and death, despite various treatments, with genetic mutations similar across lymphomas indicating a common origin.
  • A review of 18 cases shows that nearly half of patients with transformed PCMZL succumbed to the disease, particularly those diagnosed with multiple disease sites, highlighting the poor prognosis associated with this transformation.
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Article Synopsis
  • EBV-associated T/NK-cell lymphoproliferative diseases involve the clonal proliferation of EBV-positive T/NK cells, with unknown mechanisms of EBV invasion into these cells.
  • Recent studies highlight the role of exosomes from EBV-positive B cells in creating an immunosuppressive environment that aids the development of these diseases.
  • The study demonstrates that exosomes from EBV-positive B cells activate the STAT3/IL-10/PD-L1 pathway in cutaneous T-cell lymphoma cells, suggesting potential targets for improving understanding and treatment of immune evasion in EBV-T/NK-LPDs.
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We herein report a case of methotrexate-associated lymphoproliferative disorder (MTX-LPD) showing fibrin-associated large B-cell lymphoma-like heart valve lesions, and Epstein-Barr virus (EBV)-positive mucocutaneous ulcer-like cutaneous and oral mucosal lesions. MTX-LPD is a critical complication that can occur in RA patients who are treated with MTX. EBV also plays a defining or important role in LPDs.

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Rationale: Epstein-Barr virus mucocutaneous ulcers (EBVMCUs) were officially recognized as a clinicopathologic entity in the 2017 revision of the World Health Organization classification, which often occurs in the elderly or in immunosuppressive condition presented as an isolated ulcerative lesion. EBVMCUs are defined as "shallow, sharply circumscribed, mucosal or cutaneous ulcers with underlying polymorphous infiltration." It mostly involves oral mucosa, but some appear in skin or gastrointestinal tract.

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