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Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Targetoid Plaque in a Pediatric Patient: A Case Report and Review of Literature.
Pediatr Dermatol
January 2025
Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of non-Hodgkin lymphoma characterized by subcutaneous nodules, indurated plaques, erythema, and cellular infiltrates in the subcutaneous fat. Biopsies show neoplastic cells expressing cytotoxic T-cell markers and displaying moderate cytologic atypia while sparing the dermis and epidermis and showing variable degrees of necrosis, hemorrhage, and inflammatory changes. We describe a pediatric case of SPTCL in a 6-year-old boy, presenting with an unusual targetoid plaque and systemic symptoms, who showed significant improvement on systemic immunosuppressants without chemotherapy.
View Article and Find Full Text PDFSNMMI PIC case competition finalist: Subcutaneous Panniculitis-Like T-Cell Lymphoma on 18F-FDG PET/CT.
Clin Nucl Med
November 2024
Department of Radiology, University of Washington, Seattle, WA.
A 9-month-old girl was evaluated for recurrent fevers, rash, and indurated plaques, with laboratories demonstrating hyperferritinemia, hypertriglyceridemia, and pancytopenia, concerning for hemophagocytic lymphohistiocytosis. Biopsy of thigh lesion ultimately demonstrated subcutaneous panniculitis-like T-cell lymphoma. In a rare neoplasm of cytotoxic T-cells, subcutaneous panniculitis-like T-cell lymphoma presents with subcutaneous nodules in all age groups including children.
View Article and Find Full Text PDFChemo-free salvage treatment outperforms traditional chemotherapy in advanced lines of relapsed/refractory subcutaneous panniculitis-like T-cell lymphoma.
Front Immunol
December 2024
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Introduction: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma with a good prognosis, but the optimal treatment for relapsed/refractory (R/R) SPTCL has been rarely discussed.
Methods: This study aims to compare the efficacy of conventional chemotherapy and chemo-free immunomodulatory regimen for R/R SPTCL. We retrospectively reviewed the patients with first relapse or primary refractory SPTCL between September 1997 and October 2020.
Different Mutational Profiles of Subcutaneous Panniculitis-like T-cell Lymphoma and Lupus Panniculitis: An Additional Case Series.
Actas Dermosifiliogr
November 2024
Department of Pathology, Fundación Jiménez Díaz-IIS, Universidad Autónoma de Madrid, Madrid, España; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), España.
Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma with indolent behavior, mostly present in women and associated with immunological diseases whose pathogenic background is still poorly understood. SPTCL is associated with lupus erythematosus panniculitis (LEP) and histologically misdiagnosed.
Objectives: The aim of our study was to identify mutations affecting the pathogenesis of both SPTCL and LEP.
Characteristics and therapeutic outcomes of subcutaneous panniculitis-like T-cell lymphoma with and without germline HAVCR2 mutations in Thai children and adolescents.
Orphanet J Rare Dis
November 2024
Department of Pediatrics, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand.
Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma associated with hemophagocytic lymphohistiocytosis (HLH)/HLH-like systemic illnesses and germline HAVCR2 mutations. Although previous studies suggested successful treatment of SPTCL with immunosuppressive therapy (IST) without chemotherapy, IST data in pediatric SPTCL remain scarce. To explore characteristics and therapeutic outcomes, comparing IST-based and chemotherapy-based regimens in pediatric SPTCL, in this retrospective cohort study, patients with SPTCL diagnosed at age ≤20 years during 2007-2023 were enrolled from 6 hematology/oncology centers in Thailand.
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