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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. This Article Full Text (PDF) All Versions of this Article: blood-2006-09-046649v1 109/12/5477    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Matsuda, K. Articles by Koike, K. Search for Related Content PubMed PubMed Citation Articles by Matsuda, K. Articles by Koike, K. Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted September 11, 2006 Accepted February 16, 2007 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 Manabe, Seiji Kojima, and Kenichi Koike* Department of Laboratory Medicine, Shinshu University Hospital, Matsumoto, Japan Department of Hematology/Oncology, Gunma Children's Medical Center, Gunma, Japan Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan Department of Pediatrics, Niigata Cancer Center Hospital, Niigata, Japan Hamamatsu Medical Center, Hamamatsu, Japan Department of Pediatrics, Hokkaido Cancer Center, Sapporo, Japan Department of Pediatrics, Ibaraki Children's Hospital, Mito, Japan Department of Pediatrics, Fukuoka University School of Medicine, Fukuoka, Japan Department of Hematology and Oncology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan Department of Pediatrics, Yokohama City University School of Medicine, Yokohama, Japan Division of Hematology/Oncology, Saitama Children's Medical Center, Saitama, Japan Department of Pediatrics, Hyogo Collage of Medicine, Nishinomiya, Japan Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan * Corresponding author; email: koikeken{at}hsp.md.shinshu-u.ac.jp. Of 11 children with juvenile myelomonocytic leukemia (JMML) carrying RAS mutations (8, NRAS mutations; 3, KRAS2 mutations), 5 patients had a profound elevation in either or both the WBC and spleen size at diagnosis. Three patients had no or modest hepatosplenomegaly and mild leukocytosis at presentation, but subsequently showed a marked increment in spleen size with or without hematological exacerbation, for which non-intensive chemotherapy was initiated. The other 3 patients with NRAS or KRAS2 glycine to serine substitution received no chemotherapy, but hematological 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 hematological improvement, while 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 JMML patients with specific RAS mutations may have spontaneously improving disease. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Genotype-phenotype correlation in cases of juvenile myelomonocytic leukemia with clonal RAS mutations Christian Flotho, Christian P. Kratz, Eva Bergsträsser, Henrik Hasle, Jan Stary, Monika Trebo, Marry M. van den Heuvel-Eibrink, Dorota Wójcik, Marco Zecca, Franco Locatelli, and Charlotte M. Niemeyer, on behalf of the European Working Group of Myelodysplastic Syndromes in Childhood Blood 2008 111: 966-967. [Full Text] [PDF] This article has been cited by other articles: K. Foucar Myelodysplastic/Myeloproliferative Neoplasms Am J Clin Pathol, August 1, 2009; 132(2): 281 - 289. [Abstract] [Full Text] [PDF] C. Flotho, C. P. Kratz, E. Bergstrasser, H. Hasle, J. Stary, M. Trebo, M. M. van den Heuvel-Eibrink, D. Wojcik, M. Zecca, F. Locatelli, et al. Genotype-phenotype correlation in cases of juvenile myelomonocytic leukemia with clonal RAS mutations Blood, January 15, 2008; 111(2): 966 - 967. [Full Text] [PDF] K. Koike and K. Matsuda Evaluation of relationship between the genetic abnormalities and disease phenotype is required in juvenile myelomonocytic leukemia Blood, January 15, 2008; 111(2): 967 - 968. [Full Text] [PDF] C. Nava, N. Hanna, C. Michot, S. Pereira, N. Pouvreau, T. Niihori, Y. Aoki, Y. Matsubara, B. Arveiler, D. Lacombe, et al. Cardio-facio-cutaneous and Noonan syndromes due to mutations in the RAS/MAPK signalling pathway: genotype phenotype relationships and overlap with Costello syndrome J. Med. Genet., December 1, 2007; 44(12): 763 - 771. [Abstract] [Full Text] [PDF] C. Flotho, C. P. Kratz, and C. M. Niemeyer How a rare pediatric neoplasia can give important insights into biological concepts: a perspective on juvenile myelomonocytic leukemia Haematologica, November 1, 2007; 92(11): 1441 - 1446. [Full Text] [PDF] K. Matsuda, Y. Nakazawa, K. Sakashita, M. Shiohara, K. Yamauchi, and K. Koike Acquisition of loss of the wild-type NRAS locus with aggressive disease progression in a patient with juvenile myelomonocytic leukemia and a heterozygous NRAS mutation Haematologica, November 1, 2007; 92(11): 1576 - 1578. [Abstract] [Full Text] [PDF]

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