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ETV6/FLT3 fusion in a mixed-phenotype acute leukemia arising in lymph nodes in a patient with myeloproliferative neoplasm with eosinophilia

Hosseini, Niloufar ; Craddock, Kenneth J. ; Salehi-rad, Shabnam ; Brennan, Shawn ; Bailey, Denis J. ; Brandwein, Joseph M. and Porwit, Anna LU (2014) In Journal of Hematopathology 7(2). p.71-77
Abstract

The ETV6/FLT3 fusion gene has been recently reported in association with myeloproliferative neoplasm with eosinophilia (MPN-Eo) and peripheral T cell lymphoma. Favorable clinical response to a targeted FLT3 tyrosine kinase inhibitor (FLT3 TKI) was noted. Here we report a novel phenotype associated with ETV6/FLT3 rearrangement, in a 38-year-old female with presentation of progressive lymphadenopathy. Lymph node biopsy showed mixed-phenotype acute leukemia (MPAL) with expression of T cell and myeloid markers. Bone marrow morphology was consistent with MPN-Eo and no evidence of MPAL. Karyotype analysis revealed 46, XX, t(12;13)(p13;q12). ETV6/FLT3 fusion was demonstrated by fluorescence in situ hybridization. This is the first report of... (More)

The ETV6/FLT3 fusion gene has been recently reported in association with myeloproliferative neoplasm with eosinophilia (MPN-Eo) and peripheral T cell lymphoma. Favorable clinical response to a targeted FLT3 tyrosine kinase inhibitor (FLT3 TKI) was noted. Here we report a novel phenotype associated with ETV6/FLT3 rearrangement, in a 38-year-old female with presentation of progressive lymphadenopathy. Lymph node biopsy showed mixed-phenotype acute leukemia (MPAL) with expression of T cell and myeloid markers. Bone marrow morphology was consistent with MPN-Eo and no evidence of MPAL. Karyotype analysis revealed 46, XX, t(12;13)(p13;q12). ETV6/FLT3 fusion was demonstrated by fluorescence in situ hybridization. This is the first report of ETV6/FLT3 rearrangement showing a phenotype of extramedullary T/myeloid MPAL arising in the setting of MPN-Eo. We suggest addition of this entity to the WHO category of "myeloid/lymphoid neoplasms with eosinophilia," particularly given the possibility of clinical response to FLT3 TKI in MPAL, a disease usually associated with poor prognosis.

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author
; ; ; ; ; and
publishing date
type
Contribution to journal
publication status
published
subject
keywords
ETV6, FLT3, Mixed-lineage acute leukemia, Myeloproliferative disorders
in
Journal of Hematopathology
volume
7
issue
2
pages
7 pages
publisher
Springer
external identifiers
  • scopus:84903904790
DOI
10.1007/s12308-014-0203-6
language
English
LU publication?
no
id
a6bb74de-ace3-4f8f-a721-85281474e4dc
date added to LUP
2019-05-22 09:50:00
date last changed
2022-01-31 20:38:01
@article{a6bb74de-ace3-4f8f-a721-85281474e4dc,
  abstract     = {{<p>The ETV6/FLT3 fusion gene has been recently reported in association with myeloproliferative neoplasm with eosinophilia (MPN-Eo) and peripheral T cell lymphoma. Favorable clinical response to a targeted FLT3 tyrosine kinase inhibitor (FLT3 TKI) was noted. Here we report a novel phenotype associated with ETV6/FLT3 rearrangement, in a 38-year-old female with presentation of progressive lymphadenopathy. Lymph node biopsy showed mixed-phenotype acute leukemia (MPAL) with expression of T cell and myeloid markers. Bone marrow morphology was consistent with MPN-Eo and no evidence of MPAL. Karyotype analysis revealed 46, XX, t(12;13)(p13;q12). ETV6/FLT3 fusion was demonstrated by fluorescence in situ hybridization. This is the first report of ETV6/FLT3 rearrangement showing a phenotype of extramedullary T/myeloid MPAL arising in the setting of MPN-Eo. We suggest addition of this entity to the WHO category of "myeloid/lymphoid neoplasms with eosinophilia," particularly given the possibility of clinical response to FLT3 TKI in MPAL, a disease usually associated with poor prognosis.</p>}},
  author       = {{Hosseini, Niloufar and Craddock, Kenneth J. and Salehi-rad, Shabnam and Brennan, Shawn and Bailey, Denis J. and Brandwein, Joseph M. and Porwit, Anna}},
  keywords     = {{ETV6; FLT3; Mixed-lineage acute leukemia; Myeloproliferative disorders}},
  language     = {{eng}},
  number       = {{2}},
  pages        = {{71--77}},
  publisher    = {{Springer}},
  series       = {{Journal of Hematopathology}},
  title        = {{ETV6/FLT3 fusion in a mixed-phenotype acute leukemia arising in lymph nodes in a patient with myeloproliferative neoplasm with eosinophilia}},
  url          = {{http://dx.doi.org/10.1007/s12308-014-0203-6}},
  doi          = {{10.1007/s12308-014-0203-6}},
  volume       = {{7}},
  year         = {{2014}},
}