The guidelines also recommend:
• Central nervous system prophylaxis and treatment, including cranial irradiation, intrathecal chemotherapy, or high-dose systemic chemotherapy, throughout the course of therapy, from induction through maintenance, to clear leukemic cells from CNS sites that cannot be accessed by systemic chemotherapy because of the blood-brain barrier.
• Postinduction consolidation comprising drug combinations similar to those used during the induction phase, such as high-dose methotrexate, cytarabine, mercaptopurine, and l-asparaginase.
• Extended maintenance therapy for all patients (except those with mature B-cell ALL in whom relapses rarely occur beyond 12 months), typically comprising daily mercaptopurine and weekly methotrexate, often with periodic vincristine and corticosteroids, for 2 years in adults and 2-3 years in children.
• The possible inclusion of novel, immune-based agents that target specific genetic abnormalities, such as the BCR-ABL selective tyrosine kinase inhibitors for Ph-positive ALL, the anti-CD20 monoclonal antibody rituximab (Rituxan) for CD20-expression B-cell lineage ALL, and the adenosine deaminase substrate nelarabine (Arranon) for T-cell lineage ALL.
The NCCN guidelines also incorporate recommendations for minimal residual disease evaluation, provision of supportive care, and management of treatment-associated toxicities.
While the survival outcomes associated with ALL have improved dramatically among children in recent years – the cure rate with current treatment regimens is approximately 80% – the long-term prognosis for adults with the disease is poor, with cure rates of 30-40%, according to NCCN ALL guidelines panel member Dr. Daniel J. DeAngelo.
"ALL is the rarest form of adult leukemia, and we still have a lot of unanswered questions," said Dr. DeAngelo of the Dana-Farber Cancer Institute, Boston. "For this reason, adult patients with the disease should be referred to specialized cancer treatment centers and should be enrolled in clinical trials whenever possible."
Dr. Brown disclosed no relevant conflicts of interest. Dr. DeAngelo disclosed relationships with Bristol-Myers Squibb, Novartis, and Sigma-Tau Pharmaceuticals. The full list of disclosures for the NCCN ALL Guidelines Panel members can be found at http://www.nccn.org.