ETV6-FLT3–positive myeloid/lymphoid neoplasm with eosinophilia presenting in an infant: An entity distinct from JMML Journal Article
| Authors: | Spitzer, B.; Dela Cruz, F. S.; Ibanez Sanchez, G. D.; Zhang, Y.; Xiao, W.; Benayed, R.; Markova, A.; Rodriguez-Sanchez, M. I.; Bouvier, N.; Roshal, M.; Kung, A. L.; Shukla, N. |
| Article Title: | ETV6-FLT3–positive myeloid/lymphoid neoplasm with eosinophilia presenting in an infant: An entity distinct from JMML |
| 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. © 2021 by The American Society of Hematology. |
| Journal Title: | Blood Advances |
| Volume: | 5 |
| Issue: | 7 |
| ISSN: | 2473-9529 |
| Publisher: | American Society of Hematology |
| Date Published: | 2021-04-13 |
| Start Page: | 1899 |
| End Page: | 1902 |
| Language: | English |
| DOI: | 10.1182/bloodadvances.2020003699 |
| PUBMED: | 33792628 |
| PROVIDER: | scopus |
| PMCID: | PMC8045514 |
| DOI/URL: | |
| Notes: | Article -- Export Date: 3 May 2021 -- Source: Scopus |
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