Outcomes of children and young adults with T-cell acute lymphoblastic leukemia/lymphoma who present in critical status.

BACKGROUND: Patients with T-cell acute lymphoblastic leukemia and lymphoma (T-ALL/LLy) commonly present with critical features such as hyperleukocytosis and mediastinal mass, which complicates completing a diagnostic and staging workup and prevents clinical trial enrollment. PROCEDURE: Consecutive patients with T-ALL/LLy from 1999 to 2019 at the Children's Hospital of Philadelphia were analyzed for pediatric intensive care unit (PICU) admission and various high-risk features as well as clinical trial enrollment and outcome. RESULTS: We identified 153 patients newly diagnosed with T-ALL/LLy, 53 (35%) required PICU-level care within 24 hours and 73 (48%) within 7 days. Non-PICU patients had a significantly higher clinical trial enrollment rate (79.4%) versus PICU patients (56.1%, P = 0.016). Patients who enrolled on a clinical trial had similar relapse risk to those who did not enroll (relapse rate 20% vs 29%, P = 0.523). Nineteen patients were precluded from trial participation. Risk of relapse was increased for patients admitted to the PICU within 24 hours (26% vs 13%, P = 0.048). Forty-four patients with T-ALL presented with hyperleukocytosis, of which 30% relapsed versus 14% without (P = 0.082). Patients who underwent apheresis for hyperleukocytosis were statistically more likely to relapse (47% vs 15%, P = 0.007). Patients with elevated uric acid (20% vs 16%, P = 0.278), mediastinal mass (20% vs 14%, P = 0.501), or required emergent steroids (20% vs 16%, P = 0.626) had a similar relapse risk. A single second relapse patient survived. CONCLUSIONS: Almost half of T-ALL/LLy patients required PICU-level care at diagnosis, making enrollment on clinical trials challenging, but trial enrollment predicted better outcome. Physicians should balance maintaining eligibility with safety to offer patients all options.

Comparison of CALGB 10403 (Alliance) and COG AALL0232 toxicity results in young adults with acute lymphoblastic leukemia.

Adolescents and young adults (AYAs) with acute lymphoblastic leukemia have improved outcomes when treated with pediatric-inspired regimens. CALGB 10403 was the largest prospective study to evaluate the feasibility of using a pediatric regimen in AYAs with acute lymphoblastic leukemia up to 40 years of age. This article presents the toxicity events observed in the CALGB 10403 study and compares these toxicities vs those observed among AYAs treated on the same arm of the companion Children's Oncology Group (COG) AALL0232 study. Toxicities in CALGB 10403 were similar to those observed in COG AALL0232. Some grade 3 to 4 adverse events were more often reported in CALGB 10403 compared with COG AALL0232 (hyperglycemia, hyperbilirubinemia, transaminase elevation, and febrile neutropenia). Adverse events correlated with body mass index ≥30 kg/m2 and some with increasing age. The mortality rate in CALGB 10403 was low (4%) and similar to that in the COG AALL0232 trial. A caveat to this analysis is that only 39% of CALGB 10403 patients completed all planned protocol treatment. In COG AALL0232, although 74% of patients aged <18 years completed treatment, only 57% of patients aged ≥18 years completed treatment. This scenario suggests that issues associated with age and treating physician may be a factor. Due to its improved survival rates compared with historical controls, the CALGB 10403 regimen is now a standard of care. The hope is that the rate of protocol completion will increase as more familiarity is gained with this regimen. These trials were registered at www.clinicaltrials.gov as #NCT00558519 (CALGB 10403) and #NCT00075725 (COG AALL0232).