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Clonal B-cell proliferations and B-cell lymphomas may co-occur in the background of follicular helper T-cell (TFH)-derived lymphomas, most associated with EBV, which has been a well-known fact for many years in the prototypical entity "TFH lymphoma, angioimmunoblastic-type." Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (CD4 PCSM-LPD) is also a TFH-derived clonal proliferation. We searched the archives and identified four cases of CD4 PCSM-LPD with accompanying clonal B-cell proliferation (one of which showed EBER positivity), and one longstanding case of CD4 PCSM-LPD, in the background of which a B-cell lymphoma had developed. These five cases broaden experience on CD4 PCSM-LPD with accompanying B-cell proliferations and also support routine evaluation of these cases with EBV in situ hybridization, to better determine whether or not there is an association with EBV.
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http://dx.doi.org/10.1111/cup.14296 | DOI Listing |
Am J Dermatopathol
July 2025
Dermatologist, Department of Dermatopathology, Weill-Cornell Medical College and New York Presbyterian Hospital, New York, NY.
Primary cutaneous CD4+ small/medium-sized T-cell lymphoproliferative disorder (CD4+ PCSM-LPD) is distinguished from other peripheral T-cell lymphomas, particularly multifocal variants, by its indolent clinical behavior and favorable prognosis. Recent studies have suggested a T follicular helper (TFH) cell origin for these lesions; however, further studies are warranted to substantiate this hypothesis and clarify their pathogenesis. A retrospective review was conducted of all adult cases of CD4+ PCSM-LPD diagnosed at Weill Cornell Medicine between 2018 and 2024.
View Article and Find Full Text PDFCureus
April 2025
Department of Dermatology, International University of Health and Welfare Narita Hospital, Narita, JPN.
A 34-year-old man became aware of an erythematous nodule on the left nasal wing. He was treated with topical steroids and oral antibacterial agents at his local doctor, but his condition did not improve, and he was referred to our hospital. A skin biopsy revealed diffuse cellular infiltration through the dermis.
View Article and Find Full Text PDFActa Dermatovenerol Alp Pannonica Adriat
June 2025
Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder (PCSM-LPD) is characterized by a slow-growing and asymptomatic solitary plaque or tumor, usually involving the head, neck, or upper extremities. The diagnosis is established based on clinical presentation, histopathological features including pleomorphic morphology and CD4-positive immunophenotype of neoplastic T lymphocytes, and molecular analysis showing clonally rearranged T-cell receptor (TCR) genes. Plaques typical of mycosis fungoides are essentially absent.
View Article and Find Full Text PDFInt J Dermatol
February 2025
Department of Radiation Oncology, Division of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Clin Exp Dermatol
November 2024
Department of Medical and Surgical Sciences (DIMEC), University of Bologna, Bologna, Italy.
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders (PCSM-LPDs), represent a rare group of haematological diseases primarily affecting the skin. In this retrospective single-centre case series study, we aimed to investigate the demographic, clinical, therapeutic and prognostic aspects of PCSM-LPD. We collected data from cases diagnosed between 2010 and the present, employing histopathological and immunohistochemical methods following the World Health Organization criteria.
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