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      FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report

      case-report

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          Abstract

          Background

          T‐lymphoblastic lymphoma (T‐LBL) is an aggressive malignancy of T‐lymphoid precursors, rarely co‐occurring with myeloid/lymphoid neoplasms with eosinophilia (M/LNs‐Eo), with consequent rearrangement of tyrosine kinase (TK)‐related genes. The FIP1L1‐PDGFRA fusion gene is the most frequent molecular abnormality seen in eosinophilia‐associated myeloproliferative disorders, but is also present in acute myeloid leukemia (AML), T‐lymphoblastic leukemia/lymphoma (TLL), or both simultaneously. T‐LBL mainly affects children and young adults, involving lymph node, bone marrow, and thymus. It represents about 85% of all immature lymphoblastic lymphomas, whereas immature B‐cell lymphomas comprise approximately 15% of all cases of LBL.

          Case

          In this case report, we present an example of T cell lymphoblastic lymphoma with coexistent eosinophelia, treated successfully with a tyrosine‐kinase inhibitor (TKI).

          Conclusion

          FIP1L1‐PDGFRA‐positive T‐LBL and myeloproliferative disorders have excellent response to low‐dose treatment with (TKI) imatinib. Most patients achieve rapid and complete hematologic and molecular remission within weeks.

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          Most cited references11

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          A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome.

          Jan Cools, Daniel J Deangelo, Jason Gotlib … (2003)
          Idiopathic hypereosinophilic syndrome involves a prolonged state of eosinophilia associated with organ dysfunction. It is of unknown cause. Recent reports of responses to imatinib in patients with the syndrome suggested that an activated kinase such as ABL, platelet-derived growth factor receptor (PDGFR), or KIT, all of which are inhibited by imatinib, might be the cause. We treated 11 patients with the hypereosinophilic syndrome with imatinib and identified the molecular basis for the response. Nine of the 11 patients treated with imatinib had responses lasting more than three months in which the eosinophil count returned to normal. One such patient had a complex chromosomal abnormality, leading to the identification of a fusion of the Fip1-like 1 (FIP1L1) gene to the PDGFRalpha (PDGFRA) gene generated by an interstitial deletion on chromosome 4q12. FIP1L1-PDGFRalpha is a constitutively activated tyrosine kinase that transforms hematopoietic cells and is inhibited by imatinib (50 percent inhibitory concentration, 3.2 nM). The FIP1L1-PDGFRA fusion gene was subsequently detected in 9 of 16 patients with the syndrome and in 5 of the 9 patients with responses to imatinib that lasted more than three months. Relapse in one patient correlated with the appearance of a T674I mutation in PDGFRA that confers resistance to imatinib. The hypereosinophilic syndrome may result from a novel fusion tyrosine kinase - FIP1L1-PDGFRalpha - that is a consequence of an interstitial chromosomal deletion. The acquisition of a T674I resistance mutation at the time of relapse demonstrates that FIP1L1-PDGFRalpha is the target of imatinib. Our data indicate that the deletion of genetic material may result in gain-of-function fusion proteins. Copyright 2003 Massachusetts Medical Society
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            WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues

            SH Swerdlow, E Campo, NL Harris … (2017)
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              T-lymphoblastic leukemia/lymphoma.

              M. You, L. Jeffrey Medeiros, Eric Hsi (2015)
              To review important concepts from the 2013 Society for Hematopathology/European Association for Haematopathology Workshop session on T-acute lymphoblastic leukemia/T-lymphoblastic lymphoma (T-ALL/T-LBL).
              Article 0 comments Cited 49 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Salem AlShemmari:
                ORCID: https://orcid.org/0000-0002-5597-5839
                salem61@gmail.com
                Journal
                Journal ID (nlm-ta): Cancer Rep (Hoboken)
                Journal ID (iso-abbrev): Cancer Rep (Hoboken)
                Journal ID (doi): 10.1002/(ISSN)2573-8348
                Journal ID (publisher-id): CNR2
                Title: Cancer Reports
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Electronic): 2573-8348
                Publication date (Electronic): 14 December 2022
                Publication date Collection: January 2023
                Volume: 6
                Issue: 1 ( doiID: 10.1002/cnr2.v6.1 )
                Electronic Location Identifier: e1769
                Affiliations
                [ 1 ] Department of Hematology Mubarak Al‐Kabeer Hospital Jabriya Kuwait
                [ 2 ] Department of Hematopathology Kuwait Cancer Control Center Kuwait Kuwait
                [ 3 ] Department of Hematology Kuwait Cancer Control Center Kuwait Kuwait
                [ 4 ] Department of Pathology Kuwait Cancer Center Kuwait Kuwait
                [ 5 ] Department of Medicine, Faculty of Medicine Kuwait University Kuwait Kuwait
                Author notes
                [*] [* ] Correspondence

                Salem AlShemmari, Department of Hematology, Kuwait Cancer Control Center, Kuwait, Kuwait.

                Email: salem61@ 123456gmail.com

                Author information
                https://orcid.org/0000-0002-5597-5839
                Article
                Publisher ID: CNR21769
                DOI: 10.1002/cnr2.1769
                PMC ID: 9875631
                PubMed ID: 36517458
                SO-VID: 4a3c3ce9-9302-4376-a01f-25011592e672
                Copyright © © 2022 The Authors. Cancer Reports published by Wiley Periodicals LLC.
                License:

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                Date revision received : 28 November 2022
                Date received : 23 September 2021
                Date accepted : 30 November 2022
                Page count
                Figures: 5, Tables: 1, Pages: 5, Words: 2434
                Categories
                Subject: Case Report
                Subject: Case Reports
                Custom metadata
                source-schema-version-number 2.0
                cover-date January 2023
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:6.2.3 mode:remove_FC converted:25.01.2023

                Keywords: fip1l1‐pdgfra,t lymphoblastic lymphoma,tll,eosinophilia
                Data availability:
                Keywords: fip1l1‐pdgfra, t lymphoblastic lymphoma, tll, eosinophilia

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