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Author Notes:

Sunil S. Raikar (e-mail: sraikar@emory.edu)

The authors thank the cardiology and cardiothoracic surgery teams at Children's Healthcare of Atlanta for their excellent management of the patient's coarctation of aorta.

The authors declare no conflicts of interest.

Subjects:

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Hematology

Sustained Complete Molecular Remission With Imatinib Monotherapy in a Child Presenting With Blast Phase FIP1L1-PDGFRA-Associated Myeloid Neoplasm With Eosinophilia

Tools:

Journal Title:

HEMASPHERE

Volume:

Volume 4, Number 6

Publisher:

, Pages e486-e486

Type of Work:

Article | Final Publisher PDF

Abstract:

The etiology of hypereosinophilia is divided into 2 categories, clonal or reactive in origin. The clonal form typically presents as a chronic myeloproliferative neoplasm (MPN), associated with rearrangements involving PDGFRA/B, FGFR1 or with the PCM1-JAK2 fusion.1 These fusion genes result in a constitutively active tyrosine kinase, and the PDGFR-rearrangements are very sensitive to tyrosine kinase inhibitors (TKIs) such as imatinib. More rarely, these rearrangements can be seen in cases of acute myeloid leukemia (AML), T-cell lymphoblastic leukemia/lymphoma (T-ALL/T-LLy), or mixed phenotype acute leukemia (MPAL), generally associated with eosinophilia.1 Given the varied presentation, the World Health Organization now classifies these malignancies under the category of “myeloid/lymphoid neoplasms with eosinophilia (MLN-Eo) and rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2”. This disease is extremely uncommon in children and only 8 FIP1L1-PDGRFA cases have previously been described.

Copyright information:

© 2020 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association.

This is an Open Access work distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/rdf).
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