Expert Q&A: Is the Relapse Rate High for Acute T-cell Lymphoblastic Leukemia?

Expert Q&A: Is the Relapse Rate High for Acute T-cell Lymphoblastic Leukemia?

Source: Sunflower Children's Author: Xia Yu Edited by: Maixm Date: April 7, 2023

In this edition of the “Expert Q&A” section, we invited Dr. Yang Jing Tang, an associate chief physician from the Hematology/Oncology Department of the Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, to answer our questions.

Dr. Tang specializes in clinical and fundamental research on pediatric acute leukemia and cellular immunotherapy. She is particularly skilled in CAR-T clinical research for children with relapsed/refractory acute lymphoblastic leukemia, acute myeloid leukemia, and malignant lymphomas (such as Burkitt lymphoma and diffuse large B-cell lymphoma), as well as clinical research on CAR-T therapy complications.

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01

Q: A 3-year-old boy was diagnosed with leukemia at the age of 2 and is currently undergoing treatment. He is following the CCC-ALL-2020 protocol, and after 19 days, his MRD is <0.01. He is classified as low risk and has received methotrexate four times, completing the third round of maintenance therapy. The mutation and fusion genes have tested negative. However, NRAS 002524, Exon 3, c.181c>A, p.Q61K 18.37%, IKZF1 plus, and gene CNV testing showed a heterozygous deletion in the PAR1 region (IL3RA EXON1). Are these two genes indicative of poor prognosis and a high likelihood of relapse? Is it appropriate to classify him in the low-risk group?

A: If the PAR1 deletion is not IKZF1 plus, then even with IKZF1 plus, as long as there is a good response to early treatment, the prognosis remains quite good. Continue to monitor the MRD results, and if the MRD increases, consider adjusting the risk group.

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02

Q: A 10-year-old boy was diagnosed with leukemia at the age of 4 and has now completed treatment. He has T-cell lymphoblastic leukemia with a positive SIL gene, an initial white blood cell count of 400, and tested negative after thirty-three days. He has been off medication for a year and a half after three years of treatment. What is the likelihood of relapse for T-ALL?

A: T-cell relapses usually occur very early or in the early stages; relapses after 36 months are relatively rare.

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03

Q: An 11-year-old girl was diagnosed with acute lymphoblastic leukemia type L2 at the age of 9 and is currently undergoing treatment. She was diagnosed on May 17, 2021, with COMMonB, classified as medium risk; on May 19, she tested positive for the fusion gene BCR-ABL1-P190, which turned negative on June 10. She achieved bone marrow remission on May 27 and is currently receiving minor chemotherapy at home. Should she continue to take dasatinib after completing treatment? If so, for how long? Is this gene associated with a poor prognosis and a high relapse rate?

A: This fusion gene is generally associated with a less favorable prognosis. According to the CCCG protocol, dasatinib should be taken throughout the entire course of chemotherapy and stopped after treatment completion.

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04

Q: A 9-year-old girl was diagnosed with intermediate-risk B-ALL at the age of 7. Fifteen days after diagnosis, her residual cells were cleared. Due to the IKZF1 gene deletion, she is classified as medium risk, with positive results for BLNK and FLT3. The subsequent treatment process has been smooth, and she has now completed therapy. Given that her grandmother passed away from leukemia, there are concerns about the possibility of relapse despite her completion of treatment. What is the prognosis for children in this situation?

A: The IKZF1 gene deletion only has prognostic value if there is a poor response to early treatment. The fact that she turned negative for MRD after 15 days and had a smooth follow-up process, with no other high-risk factors, suggests a relatively good prognosis. However, one cannot entirely rule out the possibility of future relapse; regular follow-ups are essential.

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Editorial Director: Zhong Ruitao  

Project Management: Zuo Jia  

Typesetting: Xia Yu  

Proofreading: He Fei