Spontaneous improvement of hematologic abnormalities in patients having juvenile myelomonocytic leukemia with specific RAS mutations

Kazuyuki Matsuda, Akira Shimada, Nao Yoshida, Atsushi Ogawa, Akihiro Watanabe, Shuhei Yajima, Susumu Iizuka, Kazutoshi Koike, Fumio Yanai, Keiichiro Kawasaki, Masakatsu Yanagimachi, Akira Kikuchi, Yoshitoshi Ohtsuka, Eiko Hidaka, Kazuyoshi Yamauchi, Miyuki Tanaka, Ryu Yanagisawa, Yozo Nakazawa, Masaaki Shiohara, Atsushi ManabeSeiji Kojima, Kenichi Koike

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103 Citations (Scopus)

Abstract

Of 11 children with juvenile myelomonocytic leukemia (JMML) carrying RAS mutations (8 with NRAS mutations, 3 with KRAS2 mutations), 5 had a profound elevation in either or both the white blood cells and spleen size at diagnosis. Three patients had no or modest hepatosplenomegaly and mild leukocytosis at presentation but subsequently showed a marked increase in spleen size with or without hematologic exacerbation, for which nonintensive chemotherapy was initiated. The other three patients with NRAS or KRAS2 glycine to serine substitution received no chemotherapy, but hematologic improvement has been observed during a 2- to 4-year follow up. In the third group, all hematopoietic cell lineages analyzed had the RAS mutations at the time of hematologic improvement, whereas DNA obtained from the nails had the wild type. Additionally, numbers of circulating granulocyte-macrophage progenitors were significantly reduced during the clinical course. Thus, some patients with JMML with specific RAS mutations may have spontaneously improving disease.

Original languageEnglish
Pages (from-to)5477-5480
Number of pages4
JournalBlood
Volume109
Issue number12
DOIs
Publication statusPublished - Jun 15 2007

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ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Matsuda, K., Shimada, A., Yoshida, N., Ogawa, A., Watanabe, A., Yajima, S., Iizuka, S., Koike, K., Yanai, F., Kawasaki, K., Yanagimachi, M., Kikuchi, A., Ohtsuka, Y., Hidaka, E., Yamauchi, K., Tanaka, M., Yanagisawa, R., Nakazawa, Y., Shiohara, M., ... Koike, K. (2007). Spontaneous improvement of hematologic abnormalities in patients having juvenile myelomonocytic leukemia with specific RAS mutations. Blood, 109(12), 5477-5480. https://doi.org/10.1182/blood-2006-09-046649