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Blood, 15 June 2007, Vol. 109, No. 12, pp. 5477-5480.
Prepublished online as a Blood First Edition Paper on March 1, 2007; DOI 10.1182/blood-2006-09-046649.
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NEOPLASIA
Brief Report
Spontaneous improvement of hematologic abnormalities in patients having juvenile myelomonocytic leukemia with specific RAS mutations
Kazuyuki Matsuda1,
Akira Shimada2,
Nao Yoshida3,
Atsushi Ogawa4,
Akihiro Watanabe4,
Shuhei Yajima5,
Susumu Iizuka6,
Kazutoshi Koike7,
Fumio Yanai8,
Keiichiro Kawasaki9,
Masakatsu Yanagimachi10,
Akira Kikuchi11,
Yoshitoshi Ohtsuka12,
Eiko Hidaka1,
Kazuyoshi Yamauchi1,
Miyuki Tanaka13,
Ryu Yanagisawa13,
Yozo Nakazawa13,
Masaaki Shiohara13,
Atsushi Manabe14,
Seiji Kojima3, and
Kenichi Koike13
1 Department of Laboratory Medicine, Shinshu University Hospital, Matsumoto, Japan;
2 Department of Hematology/Oncology, Gunma Children's Medical Center, Gunma, Japan;
3 Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan;
4 Department of Pediatrics, Niigata Cancer Center Hospital, Niigata, Japan;
5 Hamamatsu Medical Center, Hamamatsu, Japan;
6 Department of Pediatrics, Hokkaido Cancer Center, Sapporo, Japan;
7 Department of Pediatrics, Ibaraki Children's Hospital, Mito, Japan;
8 Department of Pediatrics, Fukuoka University School of Medicine, Fukuoka, Japan;
9 Department of Hematology and Oncology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan;
10 Department of Pediatrics, Yokohama City University School of Medicine, Yokohama, Japan;
11 Division of Hematology/Oncology, Saitama Children's Medical Center, Saitama, Japan;
12 Department of Pediatrics, Hyogo Collage of Medicine, Nishinomiya, Japan;
13 Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan;
14 Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan
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.
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