Fast Five Quiz: B-Cell Acute Lymphoblastic Leukemia
Though more common in pediatric populations, B-cell acute lymphoblastic leukemia (B-ALL) affects both children and adults, and it has several genetic subtypes that significantly affect prognosis and treatment decisions. Despite advances in treatment protocols, resistance mechanisms remain a significant challenge. However, some genetic subtypes are associated with more favorable prognosis, and the nuances of this disease are evident in the cascading treatment algorithm recommended by the National Comprehensive Cancer Network (NCCN) for pediatric and adult cases.
What do you know about B-ALL? Check your knowledge with this quick quiz.
The treatment of B-ALL involves a complex and intensive series of steps. In the induction phase, glucocorticoids are typically used to reduce tumor burden by clearing leukemic cells from the bone marrow. However, point mutations in the NR3C1 gene generally cause resistance to glucocorticoid therapy, which can significantly affect prognosis. Mutations in the CREBBP gene also cause resistance to glucocorticoids
DHFR , FPGS , and TYMS genes have been shown to generally cause resistance to methotrexate, not glucocorticoids.
Learn more about corticosteroids in B-ALL.
In risk stratification for B-ALL, the NCCN notes that having a white blood cell count of > 30 x 109/L is considered a high-risk feature. This is consistent with European guidelines. The NCCN also states that age over 35 years is another high-risk feature for B-ALL. ETP phenotype is a high-risk feature for T-cell acute lymphoblastic leukemia rather than the B-ALL subtype. Hyperdiploidy (a molecular subtype of B-ALL with 51-65 chromosomes) is considered standard risk by the NCCN; data have shown better prognosis for pediatric patients with this subtype.
Learn more about risk stratification for B-ALL.
TKIs are an important component of induction, consolidation, and maintenance treatment for Ph+ B-ALL. They are recommended by the NCCN for use in conjunction with other drugs, such as corticosteroids, blinatumomab, and inotuzumab ozogamicin (depending on treatment phase and disease severity) and as a treatment post-HCT. They recommend continuing TKI therapy for at least 2 years after HCT, although they note that the 'optimal duration' is unknown for this population.
A recent review, however, notes that despite their success in patients with Ph+ B-ALL, approximately 25% of cases will develop resistance to TKIs. Given this possibility, the NCCN notes that clinicians should consider prior TKI intolerance, dose used, BCR::ABL1 mutations, and disease-related features when choosing a specific TKI; they specifically recommend bosutinib, dasatinib, imatinib, nilotinib, or ponatinib as options for TKI therapy.
Learn more about Ph+ B-ALL treatments.
The DUX4-rearranged subtype of B-ALL is considered to have a favorable prognosis in adolescents and young adults, compared with MEF2D-rearranged, CDX2/UBTF, and IDH1/2; these subtypes generally have inferior prognosis in the same populations (although MEF2D-rearranged has intermediate prognosis in adults). Specifically, DUX4-rearranged B-ALL has been associated with 93% event-free survival and overall survival in pediatric patients, and adolescent and young-adult patients also see longer disease-free survival after complete remission is achieved.
Learn more about B-ALL genomics.
Several genetic subtypes of B-ALL are associated with varying prognoses, and the outcomes differences seen between pediatric and adult patients can be partly explained by the different subtypes expressed by these populations. Further, new therapies have enabled high survival rates among pediatric patients with B-ALL, with long-term survival of up to 90% in this population. Prognosis for B-ALL in adolescents and adults is comparatively poor. For example, a population-based study reported the following declining survival rates with age: 74% for patients 15-19 years old, 59% for patients 20-39 years old, and 43% for those 40-59 years old. However, the researchers explained that these percentages still demonstrate improvements, as survival rates were overall lower in the 1980s and 1990s.
Learn more about B-ALL prognosis.
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Yahoo
a day ago
- Yahoo
Let's celebrate: Today is the summer solstice, the longest day of the year
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Hamilton Spectator
2 days ago
- Hamilton Spectator
‘The biggest betrayal': A year on, staff grieve Ontario Science Centre's snap closure
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NDP infrastructure critic Jennifer French also takes issue with stated reason for the closure. 'The roof managed to get through winter snow loads and a lot of rain this spring, and the only thing falling apart is the government's plan for a new science centre,' she said. The opening of the science centre at Ontario Place has already been pushed back from 2028 to 2029. A report from the auditor general late last year found that the cost estimate for building and maintaining the new science centre has increased by nearly $400 million from the government's spring 2023 business case for relocating it. Meanwhile, the government has said it will look for a space to house a temporary science centre until the new one opens. A request for proposals seeking a temporary location was released just days after the abrupt closure. It said the government was working 'expeditiously' to find an interim site and wanted it to open no later than Jan. 1, 2026. 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Unexpected explosion in the Milky Way creates a new star. Where you can see it
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