This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kudo, K. Articles by Tsukimoto, I. Search for Related Content PubMed PubMed Citation Articles by Kudo, K. Articles by Tsukimoto, I. Social Bookmarking                            What's this?

Prospective Study of a Pirarubicin, Intermediate-Dose Cytarabine, and Etoposide Regimen in Children With Down Syndrome and Acute Myeloid Leukemia: The Japanese Childhood AML Cooperative Study Group

Kazuko Kudo, Seiji Kojima, Ken Tabuchi, Hiromasa Yabe, Akio Tawa, Masue Imaizumi, Ryoji Hanada, Kazuko Hamamoto, Ryoji Kobayashi, Akira Morimoto, Hideki Nakayama, Masahiro Tsuchida, Keizo Horibe, Hisato Kigasawa, Ichiro Tsukimoto

From the Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba; Department of Pediatrics, Nagoya University, Graduate School of Medicine, Nagoya University; Clinical Research Center, National Nagoya Hospital, Nagoya; Department of Hematology, Kanagawa Children's Medical Center, Yokohama; Specialized Clinical Science, Pediatrics, Tokai University School of Medicine, Kanagawa; Department of Pediatrics, National Hospital Organization Osaka National Hospital, Osaka; Department of Hematology and Oncology, Miyagi Children's Hospital, Sendai; Saitama Children's Medical Center, Saitama; Department of Pediatrics, Hiroshima Red Cross Hospital and Atomic Bomb Survivors Hospital, Hiroshima; Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo; Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto; Department of Pediatrics, Hamanomachi Hospital, Fukuoka; Department of Pediatrics, Ibaraki Children's Hospital, Mito; and the Department of Pediatrics, Toho University School of Medicine, Tokyo, Japan

Address reprint requests to Kazuko Kudo, MD, Institute of Clinical Medicine, University of Tsukuba, 1-1-1, Tennodai, Tsukuba, Japan 305-8575; e-mail: kazukok{at}md.tsukuba.ac.jp

Purpose To evaluate a less intensive chemotherapeutic regimen specifically designed for patients with Down syndrome (DS) and acute myeloid leukemia (AML), and to determine the prognostic factors for event-free survival.

Patients and Methods Seventy-two patients with AML-DS were treated with remission induction chemotherapy consisting of pirarubicin (25 mg/m²/d for 2 days), cytarabine (100 mg/m²/d for 7 days), and etoposide (150 mg/m²/d for 3 days). Patients received four courses of intensification therapy of the same regimen. Prophylaxis for CNS leukemia was not included.

Results All but two patients were younger than 4 years, and 67 of the 72 patients (93%) were diagnosed as acute megakaryoblastic leukemia (AMKL). Seventy of the 72 patients (97.2%) achieved a complete remission (CR), and the estimated 4-year event-free survival (EFS) rate was 83% ± 9%. Nine patients relapsed, and one died as a result of pneumonia during CR. Multivariate analysis revealed that the presence of monosomy 7 was a greater risk factor of adverse outcome (odds ratio = 5.67; P = .027).

Conclusion A less intensive chemotherapeutic regimen produces excellent outcomes in standard-risk AML-DS patient. Risk-oriented therapy should be considered for future trials in AML-DS.

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

CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				A. C. Xavier, Y. Ge, and J. W. Taub Down Syndrome and Malignancies: A Unique Clinical Relationship: A Paper from the 2008 William Beaumont Hospital Symposium on Molecular Pathology J. Mol. Diagn., September 1, 2009; 11(5): 371 - 380. [Abstract] [Full Text] [PDF]

				K. R. Rabin and J. A. Whitlock Malignancy in Children with Trisomy 21 Oncologist, February 1, 2009; 14(2): 164 - 173. [Abstract] [Full Text] [PDF]