Subcutaneous Panniculitis-like T-cell Lymphoma Lacking Subcutaneous Tumor Mimicking Adult-onset Still's Disease

We herein report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) resembling adult-onset Still's disease (AOSD). A 40-year-old woman presented with a fever, erythema, and painful subcutaneous nodules on the trunk. Laboratory data and a bone marrow analysis showed hemophagocytic...

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Detalles Bibliográficos
Autores principales: Tada, Maria, Kachi, Shion, Onozawa, Masahiro, Fujieda, Yuichiro, Yoshida, Shota, Oki, Yotaro, Kamada, Kazuro, Nagai, Jun, Okada, Satomi, Kikuchi, Ryo, Hisada, Ryo, Hasegawa, Yuta, Ohigashi, Hiroyuki, Goto, Hideki, Hashimoto, Daigo, Nakazato, Shinichi, Matsuno, Yoshihiro, Teshima, Takanori, Atsumi, Tatsuya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2023
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10686735/
https://www.ncbi.nlm.nih.gov/pubmed/36927968
http://dx.doi.org/10.2169/internalmedicine.1419-22
Descripción
Sumario:We herein report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) resembling adult-onset Still's disease (AOSD). A 40-year-old woman presented with a fever, erythema, and painful subcutaneous nodules on the trunk. Laboratory data and a bone marrow analysis showed hemophagocytic syndrome. Although AOSD was suspected, based on a histopathological evaluation of the erythema, she was diagnosed with SPTCL. She was refractory to combination chemotherapy but achieved durable remission with cyclosporine monotherapy. Genetic testing revealed a homozygous HAVCR2 c.245A>G variant (rs184868814) that had caused NLRP3 inflammasome activation. SPTCL and AOSD share a pathogenesis in terms of NLRP3 inflammasome activation, so the clinical phenotype of SPTCL reasonably mimics AOSD.

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