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Blood, 1 January 2007, Vol. 109, No. 1, pp. 61-64. Prepublished online as a Blood First Edition Paper on September 7, 2006; DOI 10.1182/blood-2006-05-024828. This Article Full Text (PDF) All Versions of this Article: blood-2006-05-024828v1 109/1/61    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 David, M. Articles by Apperley, J. F. Search for Related Content PubMed PubMed Citation Articles by David, M. Articles by Apperley, J. F. Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted May 22, 2006 Accepted June 11, 2006 Durable responses to Imatinib in patients with PDGFRB fusion gene positive and BCR-ABL negative chronic myeloproliferative disorders Marianna David, Nicholas CP Cross, Sonja Burgstaller, Andrew Chase, Claire Curtis, Raymond Dang, Martine Gardembas, John M Goldman, Francis Grand, George Hughes, Francoise Huguet, Louise Lavender, Grant A McArthur, Francois X Mahon, Giorgio Massimini, Junia Melo, Philippe Rousselot, Robin J Russell-Jones, John F Seymour, Graeme Smith, Alastair Stark, Katherine Waghorn, Zariana Nikolova, and Jane F. Apperley* Department of Haematology , University of Pecs, Pecs, Hungary Wessex Regional Genetics Laboratory, Salisbury District Hospital, Salisbury, UK Department of Internal Medicine, General Hospital, Wels, Austria Department of Haematology , Dumfries General Infirmary, Dumfries, Scotland, UK Department of Haematology, CHU Angers, Angers, France Department of Haematology, Faculty of Medicine, Imperial College, London, UK Department of Haematology , West Middlesex Hospital, London, UK Department of Haematology, Hopital de Purpan, Toulouse, France Molecular Pathology Unit, Southampton General Hospital, Southampton, UK Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Australia Department of Haematology, CHR Bordeaux, Pessac, France Novartis Oncology, Basel, Switzerland Department of Haematology , Hopital St Louis, Paris, France Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Rd, London Department of Haematology , Leeds General Infirmary, Leeds, UK * Corresponding author; email: j.apperley{at}imperial.ac.uk. Fusion genes derived from the platelet-derived growth factor receptor beta (PDGFRB) or alpha (PDGFRA) play an important role in the pathogenesis of BCR-ABL negative chronic myeloproliferative disorders (CMPD). These fusion genes encode constitutively activated receptor tyrosine kinases that can be inhibited by imatinib. Twelve patients with BCR-ABL negative CMPDs and reciprocal translocations involving PDGFRB received imatinib for a median of 47 months (range 0.1-60 months). Eleven had prompt responses with rmalization of peripheral blood cell counts and disappearance of eosinophilia, 10 had complete resolution of cytogenetic abnormalities and decrease or disappearance of fusion transcripts as measured by RT-PCR. Updates were sought from 8 further patients previously described in the literature; prompt responses were described in 7 and persist in 6. Our data show that durable hematological and cytogenetic responses are achieved with imatinib in patients with PDGFRB fusion positive, BCR-ABL negative CMPDs. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Serial measurement of BCR-ABL transcripts in the peripheral blood after allogeneic stem cell transplantation for chronic myeloid leukemia: an attempt to define patients who may not require further therapy Jaspal Kaeda, Derville O'Shea, Richard M. Szydlo, Eduardo Olavarria, Francesco Dazzi, David Marin, Susan Saunders, Jamshid S. Khorashad, Nicholas C. P. Cross, John M. Goldman, and Jane F. Apperley Blood 2006 107: 4171-4176. [Abstract] [Full Text] [PDF] This article has been cited by other articles: J. Thiele Philadelphia Chromosome-Negative Chronic Myeloproliferative Disease Am J Clin Pathol, August 1, 2009; 132(2): 261 - 280. [Abstract] [Full Text] [PDF] S. Frohling and H. Dohner Chromosomal Abnormalities in Cancer N. Engl. J. Med., August 14, 2008; 359(7): 722 - 734. [Full Text] [PDF] S. Verstovsek, A. Tefferi, J. Cortes, S. O'Brien, G. Garcia-Manero, A. Pardanani, C. Akin, S. Faderl, T. Manshouri, D. Thomas, et al. Phase II Study of Dasatinib in Philadelphia Chromosome-Negative Acute and Chronic Myeloid Diseases, Including Systemic Mastocytosis Clin. Cancer Res., June 15, 2008; 14(12): 3906 - 3915. [Abstract] [Full Text] [PDF] M. C. Heinrich, H. Joensuu, G. D. Demetri, C. L. Corless, J. Apperley, J. A. Fletcher, D. Soulieres, S. Dirnhofer, A. Harlow, A. Town, et al. Phase II, Open-Label Study Evaluating the Activity of Imatinib in Treating Life-Threatening Malignancies Known to Be Associated with Imatinib-Sensitive Tyrosine Kinases Clin. Cancer Res., May 1, 2008; 14(9): 2717 - 2725. [Abstract] [Full Text] [PDF] V. Zota, P. M. Miron, B. A. Woda, A. Raza, and S. A. Wang Eosinophilia With FIP1L1-PDGFRA Fusion in a Patient With Chronic Myelomonocytic Leukemia J. Clin. Oncol., April 20, 2008; 26(12): 2040 - 2041. [Full Text] [PDF] A. Chase, F. H. Grand, and N. C. P. Cross Activity of TKI258 against primary cells and cell lines with FGFR1 fusion genes associated with the 8p11 myeloproliferative syndrome Blood, November 15, 2007; 110(10): 3729 - 3734. [Abstract] [Full Text] [PDF] A. Reiter, D. Grimwade, and N. C.P. Cross Diagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders Haematologica, September 1, 2007; 92(9): 1153 - 1158. [Full Text] [PDF] S. Fletcher and B. Bain Eosinophilic leukaemia Br. Med. Bull., April 18, 2007; (2007) ldm008v1. [Abstract] [Full Text] [PDF]

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