EJHaem · 2026
Selumetinib as an Effective Therapy of Histiocytic Sarcoma Evolving From a B-Cell Acute Lymphoblastic Leukaemia.
СтатьяНазвание и аннотация: Перевод в очереди
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Largeaud Laetitia, Syrykh Charlotte, Vial Julie, Canali Alban, Luquet Isabelle, Vergez François, Dufrechou Stéphanie, Prade Naïs, Baruchel André, Gerby Bastien, Nolla Marie, Delabesse Eric, Pasquet Marlène
Аннотация
INTRODUCTION: Histiocytic sarcoma (HS) is a rare neoplasm derived from non-Langerhans histiocytic cells, exceptionally arising from B-ALL.
METHODS: We present the case of a child with high-risk B-ALL with PAX5 P80R mutation.
RESULTS: Despite initial remission, a chemoresistant paravertebral mass was identified as HS. A shared IGK/TCRB rearrangements and PAX5 alterations between the leukaemic and histiocytic clones suggested transdifferentiation driven by PAX5. A somatic MAP2K1 mutation in the HS component prompted selumetinib treatment, leading to a rapid response.
CONCLUSION: This case underscores the role of PAX5 in lineage plasticity and highlights the potential of targeted MEK inhibition in MAPK-driven HS arising from B-ALL.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.
METHODS: We present the case of a child with high-risk B-ALL with PAX5 P80R mutation.
RESULTS: Despite initial remission, a chemoresistant paravertebral mass was identified as HS. A shared IGK/TCRB rearrangements and PAX5 alterations between the leukaemic and histiocytic clones suggested transdifferentiation driven by PAX5. A somatic MAP2K1 mutation in the HS component prompted selumetinib treatment, leading to a rapid response.
CONCLUSION: This case underscores the role of PAX5 in lineage plasticity and highlights the potential of targeted MEK inhibition in MAPK-driven HS arising from B-ALL.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.