ETV6/FLT3 fusion in a mixed-phenotype acute leukemia arising in lymph nodes in a patient with myeloproliferative neoplasm with eosinophilia
(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.
(Less)
- author
- Hosseini, Niloufar ; Craddock, Kenneth J. ; Salehi-rad, Shabnam ; Brennan, Shawn ; Bailey, Denis J. ; Brandwein, Joseph M. and Porwit, Anna LU
- publishing date
- 2014
- 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}}, }