<i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy?
The <i>MLL</i> (<i>mixed-lineage leukemia</i>) gene, located on chromosome 11q23, is involved in chromosomal translocations in a subtype of acute leukemia, which represents approximately 10% of acute lymphoblastic leukemia and 2.8% of acute myeloid leukemia cases. These trans...
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MDPI AG
2019-10-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/8/11/1341 |
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| author | Oliver Britten Denise Ragusa Sabrina Tosi Yasser Mostafa Kamel |
| author_facet | Oliver Britten Denise Ragusa Sabrina Tosi Yasser Mostafa Kamel |
| author_sort | Oliver Britten |
| collection | DOAJ |
| description | The <i>MLL</i> (<i>mixed-lineage leukemia</i>) gene, located on chromosome 11q23, is involved in chromosomal translocations in a subtype of acute leukemia, which represents approximately 10% of acute lymphoblastic leukemia and 2.8% of acute myeloid leukemia cases. These translocations form fusions with various genes, of which more than 80 partner genes for <i>MLL</i> have been identified. The most recurrent fusion partner in <i>MLL</i> rearrangements (<i>MLL</i>-r) is <i>AF4</i>, mapping at chromosome 4q21, accounting for approximately 36% of <i>MLL</i>-r leukemia and particularly prevalent in <i>MLL</i>-r acute lymphoblastic leukemia (ALL) cases (57%). <i>MLL</i>-r leukemia is associated with a sudden onset, aggressive progression, and notoriously poor prognosis in comparison to non-<i>MLL</i>-r leukemias. Despite modern chemotherapeutic interventions and the use of hematopoietic stem cell transplantations, infants, children, and adults with <i>MLL</i>-r leukemia generally have poor prognosis and response to these treatments. Based on the frequency of patients who relapse, do not achieve complete remission, or have brief event-free survival, there is a clear clinical need for a new effective therapy. In this review, we outline the current therapy options for <i>MLL</i>-r patients and the potential application of CAR-T therapy. |
| first_indexed | 2024-03-12T09:39:04Z |
| format | Article |
| id | doaj.art-5467738591e6455799c8b2591d8b7a23 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-12T09:39:04Z |
| publishDate | 2019-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-5467738591e6455799c8b2591d8b7a232023-09-02T13:21:56ZengMDPI AGCells2073-44092019-10-01811134110.3390/cells8111341cells8111341<i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy?Oliver Britten0Denise Ragusa1Sabrina Tosi2Yasser Mostafa Kamel3Division of Biosciences, College of Health and Life Sciences, Institute of Environment, Health and Societies, Brunel University London, Uxbridge UB8 3PH, UKDivision of Biosciences, College of Health and Life Sciences, Institute of Environment, Health and Societies, Brunel University London, Uxbridge UB8 3PH, UKDivision of Biosciences, College of Health and Life Sciences, Institute of Environment, Health and Societies, Brunel University London, Uxbridge UB8 3PH, UKASYS Pharmaceutical Consultants-APC Inc. 2, Bedford, Nova Scotia B4A 4L2, CanadaThe <i>MLL</i> (<i>mixed-lineage leukemia</i>) gene, located on chromosome 11q23, is involved in chromosomal translocations in a subtype of acute leukemia, which represents approximately 10% of acute lymphoblastic leukemia and 2.8% of acute myeloid leukemia cases. These translocations form fusions with various genes, of which more than 80 partner genes for <i>MLL</i> have been identified. The most recurrent fusion partner in <i>MLL</i> rearrangements (<i>MLL</i>-r) is <i>AF4</i>, mapping at chromosome 4q21, accounting for approximately 36% of <i>MLL</i>-r leukemia and particularly prevalent in <i>MLL</i>-r acute lymphoblastic leukemia (ALL) cases (57%). <i>MLL</i>-r leukemia is associated with a sudden onset, aggressive progression, and notoriously poor prognosis in comparison to non-<i>MLL</i>-r leukemias. Despite modern chemotherapeutic interventions and the use of hematopoietic stem cell transplantations, infants, children, and adults with <i>MLL</i>-r leukemia generally have poor prognosis and response to these treatments. Based on the frequency of patients who relapse, do not achieve complete remission, or have brief event-free survival, there is a clear clinical need for a new effective therapy. In this review, we outline the current therapy options for <i>MLL</i>-r patients and the potential application of CAR-T therapy.https://www.mdpi.com/2073-4409/8/11/1341<i>mll</i><i>mixed-lineage leukemia</i><i>kmt2a</i>car-t cell therapyacute leukemiachromosome translocationmll-af4 |
| spellingShingle | Oliver Britten Denise Ragusa Sabrina Tosi Yasser Mostafa Kamel <i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy? Cells <i>mll</i> <i>mixed-lineage leukemia</i> <i>kmt2a</i> car-t cell therapy acute leukemia chromosome translocation mll-af4 |
| title | <i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy? |
| title_full | <i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy? |
| title_fullStr | <i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy? |
| title_full_unstemmed | <i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy? |
| title_short | <i>MLL</i>-Rearranged Acute Leukemia with t(4;11)(q21;q23)—Current Treatment Options. Is There a Role for CAR-T Cell Therapy? |
| title_sort | i mll i rearranged acute leukemia with t 4 11 q21 q23 current treatment options is there a role for car t cell therapy |
| topic | <i>mll</i> <i>mixed-lineage leukemia</i> <i>kmt2a</i> car-t cell therapy acute leukemia chromosome translocation mll-af4 |
| url | https://www.mdpi.com/2073-4409/8/11/1341 |
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