FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report
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...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-01-01
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| Series: | Cancer Reports |
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| Online Access: | https://doi.org/10.1002/cnr2.1769 |
| _version_ | 1811169287626293248 |
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| author | Salhah Ali Yasmine Al‐Qattan Walaa Awny Abdulaziz Hamadah Karen Pinto Salem AlShemmari |
| author_facet | Salhah Ali Yasmine Al‐Qattan Walaa Awny Abdulaziz Hamadah Karen Pinto Salem AlShemmari |
| author_sort | Salhah Ali |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-10T16:39:51Z |
| format | Article |
| id | doaj.art-47ab9dac05924e278d5911ce318626a4 |
| institution | Directory Open Access Journal |
| issn | 2573-8348 |
| language | English |
| last_indexed | 2024-04-10T16:39:51Z |
| publishDate | 2023-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Cancer Reports |
| spelling | doaj.art-47ab9dac05924e278d5911ce318626a42023-02-08T07:31:30ZengWileyCancer Reports2573-83482023-01-0161n/an/a10.1002/cnr2.1769FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case reportSalhah Ali0Yasmine Al‐Qattan1Walaa Awny2Abdulaziz Hamadah3Karen Pinto4Salem AlShemmari5Department of Hematology Mubarak Al‐Kabeer Hospital Jabriya KuwaitDepartment of Hematology Mubarak Al‐Kabeer Hospital Jabriya KuwaitDepartment of Hematopathology Kuwait Cancer Control Center Kuwait KuwaitDepartment of Hematology Kuwait Cancer Control Center Kuwait KuwaitDepartment of Pathology Kuwait Cancer Center Kuwait KuwaitDepartment of Hematology Kuwait Cancer Control Center Kuwait KuwaitAbstract 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.https://doi.org/10.1002/cnr2.1769FIP1L1‐PDGFRAT lymphoblastic lymphomaTLLeosinophilia |
| spellingShingle | Salhah Ali Yasmine Al‐Qattan Walaa Awny Abdulaziz Hamadah Karen Pinto Salem AlShemmari FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report Cancer Reports FIP1L1‐PDGFRA T lymphoblastic lymphoma TLL eosinophilia |
| title | FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report |
| title_full | FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report |
| title_fullStr | FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report |
| title_full_unstemmed | FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report |
| title_short | FIP1L1‐PDGFRA fusion gene in T‐lymphoblastic lymphoma: A case report |
| title_sort | fip1l1 pdgfra fusion gene in t lymphoblastic lymphoma a case report |
| topic | FIP1L1‐PDGFRA T lymphoblastic lymphoma TLL eosinophilia |
| url | https://doi.org/10.1002/cnr2.1769 |
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