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Emergence of a prominent myeloid clone in a ZNF384-rearranged B-cell precursor acute lymphoblastic leukaemia post-corticosteroid pre-phase therapy.
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  • Alpeshkumar Kapadia,
  • Sreejesh Sreedharanunni,
  • Safal Muhammed,
  • Indrani Karmakar,
  • Sonia Rana,
  • Prashant Sharma,
  • Man Updesh Sachdeva,
  • Amita Trehan
Alpeshkumar Kapadia
Post Graduate Institute of Medical Education and Research
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Sreejesh Sreedharanunni
Post Graduate Institute of Medical Education and Research
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Safal Muhammed
Post Graduate Institute of Medical Education and Research
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Indrani Karmakar
Post Graduate Institute of Medical Education and Research
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Sonia Rana
Post Graduate Institute of Medical Education and Research
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Prashant Sharma
Post Graduate Institute of Medical Education and Research
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Man Updesh Sachdeva
Post Graduate Institute of Medical Education and Research
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Amita Trehan
Post Graduate Institute of Medical Education and Research
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Abstract

A 2-year-old girl with TCF3-ZNF384 re-arranged standard-risk B-cell precursor acute lymphoblastic leukaemia (BCP-ALL) showed an apparent lineage-switch to predominantly myeloid blasts following 7-days of corticosteroid therapy. ZNF384-re-arranged leukemias are increasingly recognized to present either as mixed-phenotype acute leukemia or as BCP-ALL with pro-B immunophenotype. Lineage switch is a rare phenomenon described at relapse or following CAR-T-cell therapy previously mostly in KMT2A re-arranged leukemias; and now, also in ZNF384-rearranged patients. It is previously unreported in any patient after pre-phase corticosteroid therapy. Hematologists should be aware of this distinct emerging entity among leukemias with multi-lineage differentiation potential.

Peer review status:Published

19 Jul 2020Published in Pediatric Blood & Cancer. 10.1002/pbc.28513