Atypical Gamma‐Delta‐Positive T‐Cell Lymphoproliferation With Clinical Features of Lymphomatoid Papulosis in Three Children

• Published in: JEADV clinical practice Vol. 4; no. 4; pp. 883 - 886
• Main Authors: Madeleine, Lachance, Caroline, Ram‐Wolff, Bernard, Delisle, Nina, Sigg, Pauline, Bataille, Emmanuelle, Bourrat, Maëlle, Dumont, Gilles, Battesti, Alizée, Bozonnat, Baptiste, Louveau, Samia, Mourah, Hélène, Moins‐Teisserenc, Martine, Bagot, Laurence, Lamant, Anne, Croue, Sophie, Michalak, Ismael, BAH, Werner, Kempf, Maxime, Battistella, Adèle, De Masson
• Format: Journal Article
• Language: English
• Published: Wiley 01.09.2025
• Subjects: cutaneous lymphomas; cutaneous lymphoproliferations; lymphomatoid papulosis
• ISSN: 2768-6566; 2768-6566
• DOI: 10.1002/jvc2.70085

ABSTRACT Primary cutaneous gamma‐delta T‐cell lymphoma has been described as an aggressive entity with a poor prognosis. However, gamma‐delta T‐cell receptor expression has been described in various types of skin lymphoproliferations. Paediatric cases of LyP are increasingly recognized, but paediatric LyP with a gamma‐delta phenotype have been rarely described. We report three paediatric patients with indolent gamma‐delta lymphoproliferation, with a relapsing‐remitting course evoking LyP. These three cases emphasize that TCR gamma‐delta expression in a lymphoproliferation is not a synonym of gamma‐delta lymphoma. Indeed, these cases raise the question of a paediatric variant of CD30‐negative lymphomatoid papulosis with histological features of atypical gamma‐delta‐positive T‐cell lymphoproliferation and underline the necessity of cautious clinico‐histological correlation when facing a gamma‐delta lymphoproliferation to avoid overtreatment. Capsule summary We report three paediatric patients with indolent gamma‐delta lymphoproliferation, with a relapsing‐remitting course evoking LyP. Skin biopsies revealed CD8+ and/or CD4+ T cells, without large CD30+ cells, with conserved expression of CD7 and the expression of at least one cytotoxic marker. T‐cells expressed TCR delta, and there was an important T‐cell clone by PCR in all patients. PET‐CT scan and blood tests were normal in all patients. There was no aggressive evolution after a mean follow‐up of 2 years.