International Journal of Hematology

DOI: 10.1007/s12185-018-2492-5 Pages: 1-5

Clinical characteristics of pediatric patients with myeloid sarcoma without bone marrow involvement in Japan

1. Leukemia/Lymphoma Committee of Japanese Society of Pediatric Hematology and Oncology

2. Shiga University of Medical Science, Department of Pediatrics

3. Kyoto Prefectural University of Medicine, Department of Pediatrics

4. Kyushu University, Department of Pediatrics, Graduate School of Medicine

5. Nagoya Medical Center, Department of Pediatrics, National Hospital Organization

6. Niigata Cancer Center Hospital, Department of Pediatrics

7. Anjo Kosei Hospital, Department of Pediatrics

8. Juntendo University Hospital, Department of Pediatrics

9. Tottori University Hospital, Department of Pediatrics

10. Hyogo Children’s Medical Center, Department of Hematology/Oncology

11. Kansai Medical University, Department of Pediatrics

12. Kyoto University, Department of Human Health Sciences, Graduate School of Medicine

13. Tokyo Medical and Dental University Graduate School of Medical and Dental Sciences, Department of Pediatrics and Developmental Biology

14. Saitama Children’s Medical Center, Department of Hematology/Oncology

15. St. Luke’s International Hospital, Department of Pediatrics

16. Kyorin University Faculty of Health Sciences, Department of Medical Technology

17. Ehime Prefectural Central Hospital, Department of Pediatrics

Correspondence to:
Takashi Taga
Tel: +81-77-548-2228
Email: ttaga@belle.shiga-med.ac.jp

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Abstract

Myeloid sarcoma (MS) is a rare neoplastic condition that is often described in association with acute myeloid leukemia (AML). MS in childhood has received little attention, particularly in Japan. We carried out a nationwide retrospective analysis of Japanese children diagnosed with MS without bone marrow involvement. Inclusion criteria were diagnosis of MS at younger than 20 years of age between January 1, 2000 and December 31, 2013. There was a predominance of males (8:2), and the median age at MS diagnosis was 4 years. Sites of involvement varied and included skin (n = 3), head and/or neck (n = 2), and multiple sites (n = 2). Karyotypes were evaluated in seven patients, with one individual carrying t(8;21) and t(9;11). Four patients developed bone marrow involvement 2–55 months after diagnosis of MS. All patients received chemotherapy for de novo AML and two individuals received HSCT in first remission. Seven of ten patients survived for 50–152 months (median, 93 months) without disease after initial chemotherapy. This retrospective study confirmed that pediatric MS without bone marrow involvement in Japan is a very rare disease. MS patients responded favorably to therapies for de novo AML, and HSCT in first remission was not indicated for all patients.

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