• Media type: E-Article
  • Title: ETV6-FLT3–positive myeloid/lymphoid neoplasm with eosinophilia presenting in an infant: an entity distinct from JMML
  • Contributor: Spitzer, Barbara; Dela Cruz, Filemon S.; Ibanez Sanchez, Glorymar D.; Zhang, Yanming; Xiao, Wenbin; Benayed, Ryma; Markova, Alina; Rodriguez-Sanchez, M. Irene; Bouvier, Nancy; Roshal, Mikhail; Kung, Andrew L.; Shukla, Neerav
  • Published: American Society of Hematology, 2021
  • Published in: Blood Advances, 5 (2021) 7, Seite 1899-1902
  • Language: English
  • DOI: 10.1182/bloodadvances.2020003699
  • ISSN: 2473-9529; 2473-9537
  • Origination:
  • Footnote:
  • Description: Abstract Myeloid/lymphoid neoplasm with eosinophilia (MLN-Eo) is a World Health Organization (WHO) established category of hematologic malignancies primarily arising in adults. We discuss an 8-month-old infant who presented with clinical features similar to those of juvenile myelomonocytic leukemia (JMML) but who was diagnosed with MLN-Eo driven by an ETV6-FLT3 fusion. Results of patient-derived leukemia ex vivo studies demonstrated increased sensitivity to type I FLT3 inhibitors as compared with type II inhibitors. Treatment with the type I inhibitor gilteritinib resulted in complete immunophenotypic and cytogenetic remission. This patient subsequently underwent a hematopoietic stem cell transplant and remains in complete remission 1 year later. This is the youngest patient reported with an ETV6-FLT3 fusion and adds to the mounting reports of FLT3-rearranged MLN-Eo, supporting its addition to the WHO classification. Furthermore, this case highlights the clinical utility of ex vivo drug testing of targeted therapies.
  • Access State: Open Access