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Clinical course of juvenile myelomonocytic leukemia in the blast crisis phase treated by acute myeloid leukemia-oriented chemotherapy and allogeneic hematopoietic stem cell transplantation

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Abstract

Juvenile myelomonocytic leukemia (JMML) is a mixed myeloproliferative and myelodysplastic disorder that occurs in early childhood. The clinical course of JMML is highly variable. A third of patients follow a relatively indolent course, although approximately 15 % cases are thought to develop acute myeloid leukemia, referred to as blast crisis. The etiology and clinical characteristics of blast crisis remain unclear. We document the case of a 27-month-old boy who presented with hepatosplenomegaly, skin rash, and lymphadenopathy. An initial diagnosis of acute erythroid leukemia was made according to the French–American–British classification. Following estimation of hypersensitivity to GM-CSF and genetic analysis of PTPN11, he was diagnosed with JMML in the blast crisis phase. Although he had several poor prognostic factors, including monosomy 7 and high HbF percentage, he achieved partial remission after treatment with acute myeloid leukemia-oriented chemotherapy followed by allogeneic hematopoietic stem cell transplantation. He has been in complete remission for over 6 years.

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Acknowledgments

We would like to thank Yoshitoshi Otsuka (Department of Pediatrics, Hyogo College of Medicine) for analyzing the hypersensitivity to GM-CSF by in vitro colony assay and Yoshihiro Mine (Kinki University Life Science Research Institute) for helping sequence analysis of PTPN11 gene.

Conflict of interest

The authors declare that they have no competing interests.

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Authors and Affiliations

  1. Department of Pediatrics, Kinki University School of Medicine, 377-2, Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan

    Satoshi Ueda, Naoki Sakata & Tsukasa Takemura

  2. Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Hideki Muramatsu, Hirotoshi Sakaguchi, Xinan Wang, Yinyan Xu & Seiji Kojima

  3. Department of Medical laboratory, Kinki University School of Medicine, Osaka, Japan

    Toshihiro Yamaguchi

  4. Department of Pediatrics, Ryukyu University School of Medicine, Okinawa, Japan

    Takeshi Higa

Authors
  1. Satoshi Ueda
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  2. Naoki Sakata
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  3. Hideki Muramatsu
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  4. Hirotoshi Sakaguchi
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  5. Xinan Wang
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  6. Yinyan Xu
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  7. Seiji Kojima
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  10. Tsukasa Takemura
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Correspondence to Naoki Sakata.

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Ueda, S., Sakata, N., Muramatsu, H. et al. Clinical course of juvenile myelomonocytic leukemia in the blast crisis phase treated by acute myeloid leukemia-oriented chemotherapy and allogeneic hematopoietic stem cell transplantation. Int J Hematol 100, 502–506 (2014). https://doi.org/10.1007/s12185-014-1638-3

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  • DOI: https://doi.org/10.1007/s12185-014-1638-3

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