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Treatment With All-Trans Retinoic Acid and Anthracycline Monochemotherapy for Children With Acute Promyelocytic Leukemia: A Multicenter Study by the PETHEMA Group

From the Hospital Universitario Materno-Infantil Vall D'Hebron, Barcelona; Hospital Niño Jesús, Madrid; Hospital Universitario La Fe, Valencia; Hospital Universitario La Fe (Infantil), Valencia; Hospital La Paz (Infantil), Madrid; Hospital Universitario Virgen del Rocío, Sevilla; Hospital Universitario Virgen de la Arrixaca (Pediatría), Murcia; Hospital Materno-Infantil de Las Palmas; Hospital Son Dureta, Palma de Mallorca; Hospital Juan Canalejo, La Coruña; Hospital Reina Sofía, Córdoba; Hospital Universitario Marqués de Valdecilla, Santander; Hospital 12 de Octubre, Madrid; Hospital Montecelo, Pontevedra; Hospital Universitario Puerta del Mar, Cádiz; Hospital Insular de Las Palmas; and Hospital Universitario de Salamanca, Spain

Address reprint requests to Miguel A. Sanz, Servicio de Hematología, Hospital Universitario La Fe, Avenida Campanar 21, 46009 Valencia, Spain; e-mail: msanz{at}uv.es

PURPOSE: To analyze the simultaneous combination of all-trans retinoic acid (ATRA) and anthracycline monochemotherapy for children with acute promyelocytic leukemia (APL).

PATIENTS AND METHODS: Since November 1996, 66 children (younger than 18 years) with genetically proven APL received induction therapy with ATRA and idarubicin. Consolidation therapy consisted of three courses of anthracycline monochemotherapy. After November 1999, patients with intermediate and high risk of relapse received consolidation therapy with ATRA and slightly reinforced doses of idarubicin. Maintenance therapy consisted of ATRA and low-dose mercaptopurine and methotrexate.

RESULTS: Thirty-nine girls (59%) and 27 boys (41%) were included in this study. The WBC count at presentation was more than 10 x 10⁹/L in 26 patients (39%). Sixty-one children (92%) achieved complete remission (CR). Early deaths from hemorrhage and retinoic acid syndrome occurred in three patients and two patients, respectively. Toxicity was manageable during consolidation and maintenance therapy. No deaths in CR, clinical cardiomyotoxicity, or secondary malignancy occurred. Two patients had molecular persistence at the end of consolidation. Three clinical relapses and two molecular relapses were also observed. Apart from one molecular relapse, all these events occurred among children with hyperleukocytosis. The 5-year cumulative incidence of relapse was 17%, whereas disease-free and overall survival rates were 82% and 87%, respectively.

CONCLUSION: A high incidence of hyperleukocytosis in children with APL was confirmed. Besides low toxicity and a high degree of compliance, a risk-adapted therapy combining ATRA and anthracycline monochemotherapy showed an antileukemic efficacy comparable to those previously reported with other chemotherapy combinations in children.

Presented in part at the 45th Annual Meeting of the American Society of Hematology, San Diego, California, December 3-7, 2003.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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