While outcomes for children with T-cell acute lymphoblastic leukemia
(T-ALL) and T-lymphoblastic lymphoma (T-LL) have improved significantly
with contemporary therapy, outcomes for patients with relapsed or
refractory (r/r) disease remain dismal. Improved risk stratification and
the incorporation of novel therapeutics have the potential to improve
outcomes further in T-ALL/T-LL by limiting relapse risk and improving
salvage rates for those with r/r disease. In this review we will discuss
the challenges and new opportunities for improved risk stratification in
T-ALL and T-LL. We will further discuss the recent incorporation of the
novel therapeutics nelarabine and bortezomib into front-line therapy for
children with T-ALL and T-LL. Finally, we will address new classes of
targeted small molecule inhibitors, immunotherapeutics, and chimeric
antigen receptor T-cell therapies under investigation in r/r T-ALL and
T-LL.
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