How I treat adults with relapsed or refractory Philadelphia chromosome-negative acute lymphoblastic leukemia

The long-term prognosis of adult patients with relapsed Philadelphia chromosome-negative acute lymphoblastic lymphoma (ALL) is poor. Allogeneic stem cell transplant in second remission is the only curative approach and is the goal when feasible. There is no standard chemotherapy regimen for relapsed disease, although a few agents are approved for use in this setting. The bispecific CD19-directed CD3 T-cell engager, blinatumomab, has recently been granted accelerated approval by the US Food and Drug Administration for relapsed or refractory disease of B-cell lineage. For patients with relapsed T-cell ALL, nelarabine is available. Liposomal vincristine is also approved for relapsed disease. When selecting combination chemotherapy salvage options, evaluation of the prior treatment and timing of relapse informs treatment decisions. Monoclonal and cellular investigational therapies are quite promising and should be explored in the appropriate patient.