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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 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 Citation Map 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 Neoplasia Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  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. 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: 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] L. Motoda, M. Osato, N. Yamashita, B. Jacob, L. Q. Chen, M. Yanagida, H. Ida, H.-J. Wee, A. X. Sun, I. Taniuchi, et al. Runx1 Protects Hematopoietic Stem/Progenitor Cells from Oncogenic Insult Stem Cells, December 1, 2007; 25(12): 2976 - 2986. [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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