Abstract
Improvement in the outcomes associated with acute lymphoblastic leukemia (ALL) in Down syndrome (DS-ALL) has been delayed. However, as the clinical characteristics of DS-ALL, including leukemogenesis, are elucidated, strategies for improving the outcomes are being considered and implemented. As host side problems, infectious complications and complication deaths due to immunodeficiency and mucosal disorders are problematic. Close control of infection using prophylactic antibiotics and intravenous immunoglobulin replacement should assist in overcoming these problems. DS-ALL is commonly associated with a poor prognosis for gene abnormality, similar to Ph-like ALL. While selecting appropriate treatment for DS-ALL, minimal residual disease (MRD) is determined to assess the disease status and calculate the risk. DS-ALL is considered as a good indication for immunotherapy, such as inotuzumab ozogamicin, blinatumomab, and chimeric antigen receptor T cell therapy, because of less adverse events than those of anticancer drugs. CRLF2-JAK abnormalities are frequently observed in DS-ALL, and specific therapies targeting them are also being developed. As mentioned above, it is thought that improvements in the treatment of DS-ALL outcomes will lead to similar improvements in other ALL. This review will point out the current and future direction of DS-ALL to clinicians treating DS-ALL and those doing research on DS-ALL.
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Okamoto, Y. (2020). Acute Lymphoblastic Leukemia in Down Syndrome. In: Kato, M. (eds) Pediatric Acute Lymphoblastic Leukemia. Springer, Singapore. https://doi.org/10.1007/978-981-15-0548-5_11
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DOI: https://doi.org/10.1007/978-981-15-0548-5_11
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