The Many Faces of Philadelphia: A Mature T-Cell Lymphoma with Variant Philadelphia-Translocation and Duplication of the Philadelphia Chromosome.
| Title: | The Many Faces of Philadelphia: A Mature T-Cell Lymphoma with Variant Philadelphia-Translocation and Duplication of the Philadelphia Chromosome. |
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| Authors: | Vida, Livia1 (AUTHOR) vida.livia@pte.hu, Horváth, Bálint1 (AUTHOR), Egyed, Miklós2 (AUTHOR), Kajtár, Béla1 (AUTHOR) kajtar.bela@pte.hu, Alizadeh, Hussain3 (AUTHOR) |
| Source: | Hematology Reports. Feb2025, Vol. 17 Issue 1, p1. 9p. |
| Subject Terms: | *T-cell lymphoma, *PHILADELPHIA chromosome, *CHROMOSOME duplication, *OVERALL survival, *GENETIC testing |
| Abstract: | Background: T-cell prolymphocytic leukemia (T-PLL) is a rare mature T-cell lymphoma that is usually associated with poor prognosis and short overall survival. Methods: We present a case of a 61-year-old woman presenting with T-PLL and the leukemic cells harboring BCR::ABL1 (BCR—breakpoint cluster region; ABL1—ABL protooncogene 1) fusion transcripts as the result of a variant of t(9;22)(q34;q11) called Philadelphia translocation: t(9;22;18)(q34;q11;q21). Sequencing revealed a rare BCR transcript with an exon 6 breakpoint corresponding to e6a2 transcripts, which has thus far been reported in only 26 cases of leukemias. Results: After 9 months of follow-up, the disease progressed and required treatment. Following alemtuzumab and chemotherapy, a short course of imatinib therapy stabilized the disease for six months, which was followed by progression and the demise of the patient. Conclusions: To the best of our knowledge, this is the first report of a mature T-cell lymphoma with a variant Philadelphia-translocation and a very rare type of BCR::ABL1 transcript. This case highlights the importance of comprehensive genetic testing of malignancies, as abnormal molecular pathways may be uncovered that may be specifically targeted by drugs. [ABSTRACT FROM AUTHOR] |
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| Items | – Name: Title Label: Title Group: Ti Data: The Many Faces of Philadelphia: A Mature T-Cell Lymphoma with Variant Philadelphia-Translocation and Duplication of the Philadelphia Chromosome. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Vida%2C+Livia%22">Vida, Livia</searchLink><relatesTo>1</relatesTo> (AUTHOR)<i> vida.livia@pte.hu</i><br /><searchLink fieldCode="AR" term="%22Horváth%2C+Bálint%22">Horváth, Bálint</searchLink><relatesTo>1</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Egyed%2C+Miklós%22">Egyed, Miklós</searchLink><relatesTo>2</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Kajtár%2C+Béla%22">Kajtár, Béla</searchLink><relatesTo>1</relatesTo> (AUTHOR)<i> kajtar.bela@pte.hu</i><br /><searchLink fieldCode="AR" term="%22Alizadeh%2C+Hussain%22">Alizadeh, Hussain</searchLink><relatesTo>3</relatesTo> (AUTHOR) – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22Hematology+Reports%22">Hematology Reports</searchLink>. Feb2025, Vol. 17 Issue 1, p1. 9p. – Name: Subject Label: Subject Terms Group: Su Data: *<searchLink fieldCode="DE" term="%22T-cell+lymphoma%22">T-cell lymphoma</searchLink><br />*<searchLink fieldCode="DE" term="%22PHILADELPHIA+chromosome%22">PHILADELPHIA chromosome</searchLink><br />*<searchLink fieldCode="DE" term="%22CHROMOSOME+duplication%22">CHROMOSOME duplication</searchLink><br />*<searchLink fieldCode="DE" term="%22OVERALL+survival%22">OVERALL survival</searchLink><br />*<searchLink fieldCode="DE" term="%22GENETIC+testing%22">GENETIC testing</searchLink> – Name: Abstract Label: Abstract Group: Ab Data: Background: T-cell prolymphocytic leukemia (T-PLL) is a rare mature T-cell lymphoma that is usually associated with poor prognosis and short overall survival. Methods: We present a case of a 61-year-old woman presenting with T-PLL and the leukemic cells harboring BCR::ABL1 (BCR—breakpoint cluster region; ABL1—ABL protooncogene 1) fusion transcripts as the result of a variant of t(9;22)(q34;q11) called Philadelphia translocation: t(9;22;18)(q34;q11;q21). Sequencing revealed a rare BCR transcript with an exon 6 breakpoint corresponding to e6a2 transcripts, which has thus far been reported in only 26 cases of leukemias. Results: After 9 months of follow-up, the disease progressed and required treatment. Following alemtuzumab and chemotherapy, a short course of imatinib therapy stabilized the disease for six months, which was followed by progression and the demise of the patient. Conclusions: To the best of our knowledge, this is the first report of a mature T-cell lymphoma with a variant Philadelphia-translocation and a very rare type of BCR::ABL1 transcript. This case highlights the importance of comprehensive genetic testing of malignancies, as abnormal molecular pathways may be uncovered that may be specifically targeted by drugs. [ABSTRACT FROM AUTHOR] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of Hematology Reports is the property of MDPI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.3390/hematolrep17010001 Languages: – Code: eng Text: English PhysicalDescription: Pagination: PageCount: 9 StartPage: 1 Subjects: – SubjectFull: T-cell lymphoma Type: general – SubjectFull: PHILADELPHIA chromosome Type: general – SubjectFull: CHROMOSOME duplication Type: general – SubjectFull: OVERALL survival Type: general – SubjectFull: GENETIC testing Type: general Titles: – TitleFull: The Many Faces of Philadelphia: A Mature T-Cell Lymphoma with Variant Philadelphia-Translocation and Duplication of the Philadelphia Chromosome. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Vida, Livia – PersonEntity: Name: NameFull: Horváth, Bálint – PersonEntity: Name: NameFull: Egyed, Miklós – PersonEntity: Name: NameFull: Kajtár, Béla – PersonEntity: Name: NameFull: Alizadeh, Hussain IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 02 Text: Feb2025 Type: published Y: 2025 Identifiers: – Type: issn-print Value: 20388322 Numbering: – Type: volume Value: 17 – Type: issue Value: 1 Titles: – TitleFull: Hematology Reports Type: main |
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