Researchers affiliated with the European LeukemiaNet have published guidelines for the management of acute promyelocytic leukemia (APL). These guidelines were published in an early online manuscript in Blood on September 23, 2008.¹

Acute promyelocytic leukemia is a relatively rare leukemia associated with only 700-800 new cases per year in the United States. However, this is a highly curable leukemia if managed properly due to the efficacy of all-trans retinoic acid (ATRA, Vesanoid®) and arsenic trioxide (ATO, Trisenox®) and to the recognition and treatment of complications unique to this disease. Acute promyelocytic leukemia is associated with the cytogenetic transformation of (15;17)(q22;q12), which results in the PML/RARA fusion abnormality, where ATRA specifically works.

One of the things stressed in the guidelines was the importance of early treatment with ATRA based on morphology while awaiting the results of cytogenetic and molecular studies for confirmation of the diagnosis. Delay in treatment can have adverse consequences. Guidelines also stress early intervention with fresh plasma and platelet transfusions to prevent bleeding. Hyperleucocytosis should prompt immediate treatment with ATRA.

The usual induction regimen consists of ATRA and an anthracycline, although there is emerging evidence that ATO can be combined with ATRA for induction therapy with good results. However, ATO provides excellent control for patients who fail initial therapy. Molecular monitoring is very important, and minimal residual disease should be treated aggressively. For patients who fail initial therapy, autologous and allogeneic stem cell transplants in second remission are indicated. Systemic and intrathecal therapy are given for CNS relapses.

Comments: These guidelines should be helpful for treating APL. However, patients should be treated on cooperative group protocols whenever possible.

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Arsenic Trioxide is Best Therapy for Relapsed Acute Promyelocytic Leukemia (04/24/2003)

Long Term Follow-up Confirms Importance of ATRA in Curing Acute Promyelocytic Leukemia 

(12/05/2002)

Reference:

¹ Sanz MA, Grimwade D, Tallman MS, et al. Guidelines on the management of acute promyelocytic leukemia: Recommendations from an expert panel on behalf of the European LeukemiaNet. Blood [early online publication]. September 23, 2008.