EJHaem · 2026
Felty Syndrome-Associated Clonal Cytotoxic T-Cell Disease With Terminal Aggressive Progression: Shared STAT3 and TET2 Mutations Across a Prolonged Disease Course.
СтатьяНазвание и аннотация: Перевод в очереди
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Saito Kei, Yokohama Akihiko, Kwan Ee Oh, Sorimachi Momoka, Imamura Kenji, Kobayashi Nobuhiko, Miyazawa Yuri, Ogawa Yoshiyuki, Saito Takayuki, Shirakura Takahiro, Nobusawa Sumihito, Handa Hiroshi
Аннотация
INTRODUCTION: Biological overlap between Felty syndrome (FS) and clonal cytotoxic T-cell disorders is increasingly recognized, but aggressive progression is uncommon.
METHODS: We investigated an autopsy-confirmed case of terminal aggressive clonal T-cell disease that developed after a 4-year clinical course diagnosed as FS using serial immunophenotypic and molecular analyses.
RESULTS: Evolving γδ T-cell populations, TCR clonality, shared STAT3 and TET2 mutations, and a newly emergent complex karyotype supported clonal continuity and terminal aggressive progression.
CONCLUSION: This case provides clinicopathological evidence of long-term evolution in FS-associated clonal cytotoxic T-cell disease.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.
METHODS: We investigated an autopsy-confirmed case of terminal aggressive clonal T-cell disease that developed after a 4-year clinical course diagnosed as FS using serial immunophenotypic and molecular analyses.
RESULTS: Evolving γδ T-cell populations, TCR clonality, shared STAT3 and TET2 mutations, and a newly emergent complex karyotype supported clonal continuity and terminal aggressive progression.
CONCLUSION: This case provides clinicopathological evidence of long-term evolution in FS-associated clonal cytotoxic T-cell disease.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.