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Blood, 1 August 2008, Vol. 112, No. 3, pp. 844-847. Prepublished online as a Blood First Edition Paper on June 2, 2008; DOI 10.1182/blood-2008-01-135897. This Article Full Text (PDF) Supplemental Methods and Table All Versions of this Article: blood-2008-01-135897v1 112/3/844    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 Vannucchi, A. M. Articles by Barbui, T. Search for Related Content PubMed PubMed Citation Articles by Vannucchi, A. M. Articles by Barbui, T. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted January 28, 2008 Accepted May 9, 2008 Characteristics and clinical correlates of MPL 515W>L/K mutation in essential thrombocythemia Alessandro M. Vannucchi*, Elisabetta Antonioli, Paola Guglielmelli, Alessandro Pancrazzi, Vittoria Guerini, Giovanni Barosi, Marco Ruggeri, Giorgina Specchia, Francesco Lo Coco, Federica Delaini, Laura Villani, Silvia Finotto, Emanuele Ammatuna, Renato Alterini, Valentina Carrai, Gloria Capaccioli, Simonetta Di Lollo, Vincenzo Liso, Alessandro Rambaldi, Alberto Bosi, and Tiziano Barbui Ematologia, Universita degli Studi, Firenze, Italy Divisione di Ematologia, Ospedali Riuniti, Bergamo, Italy Laboratorio di Epidemiologia Clinica, IRCCS Policlinico S. Matteo, Pavia, Italy Dipartimento di Ematologia, Ospedale San Bortolo, Vicenza, Italy Dipartimento di Ematologia, Universita degli Studi, Bari, Italy Dipartimento di Biopatologia, Universita Tor Vergata, Roma, Italy Dipartimento di Oncologia, Universita degli Studi, Firenze, Italy Istituto Toscano Tumori, Firenze, Italy * Corresponding author; email: amvannucchi{at}unifi.it. Among 994 patients with essential thrombocythemia (ET) who were genotyped for MPLW515L/K mutation 30 mutant patients (3.0%) were identified, 8 of whom also displayed the JAK2V671F mutation. MPLW515L/K patients presented lower hemoglobin level and higher platelet count than MPLwt; these differences were highly significant compared to MPLwt/JAK2V617F-positive patients. Reduced hemoglobin and increased platelet level were preferentially associated with W515L and W515K allele, respectively. MPL mutation was a significant risk factor for microvessel disturbances, suggesting platelet hyper-reactivity associated with constitutively active MPL; arterial thromboses were increased only in comparison to MPLwt/JAK2wt patients. MPLW515L/K mutant patients presented reduced total and erythroid bone marrow cellularity, whereas number of megakaryocytes, megakaryocytic clusters and small-sized megakaryocytes were all significantly increased. These data indicate that MPLW515L/K mutation does not define a distinct phenotype in ET, although some differences depended on the JAK2V617F mutational status of the counterpart. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: P. J. Campbell, D. Bareford, W. N. Erber, B. S. Wilkins, P. Wright, G. Buck, K. Wheatley, C. N. Harrison, and A. R. Green Reticulin Accumulation in Essential Thrombocythemia: Prognostic Significance and Relationship to Therapy J. Clin. Oncol., June 20, 2009; 27(18): 2991 - 2999. [Abstract] [Full Text] [PDF] A. M. Vannucchi, P. Guglielmelli, and A. Tefferi Advances in Understanding and Management of Myeloproliferative Neoplasms CA Cancer J Clin, May 1, 2009; 59(3): 171 - 191. [Abstract] [Full Text] [PDF] S. Schnittger, U. Bacher, C. Haferlach, T. Geer, P. Muller, J. Mittermuller, P. Petrides, R. Schlag, R. Sandner, J. Selbach, et al. Detection of JAK2 exon 12 mutations in 15 patients with JAK2V617F negative polycythemia vera Haematologica, March 1, 2009; 94(3): 414 - 418. [Abstract] [Full Text] [PDF] R. Tiedt, J. Coers, S. Ziegler, A. Wiestner, H. Hao-Shen, C. Bornmann, J. Schenkel, S. Karakhanova, F. J. de Sauvage, C. W. Jackson, et al. Pronounced thrombocytosis in transgenic mice expressing reduced levels of Mpl in platelets and terminally differentiated megakaryocytes Blood, February 19, 2009; 113(8): 1768 - 1777. [Abstract] [Full Text] [PDF] S. Schnittger, U. Bacher, C. Haferlach, D. Beelen, P. Bojko, D. Burkle, R. Dengler, A. Distelrath, M. Eckart, R. Eckert, et al. Characterization of 35 new cases with four different MPLW515 mutations and essential thrombocytosis or primary myelofibrosis Haematologica, January 1, 2009; 94(1): 141 - 144. [Full Text] [PDF] P. Guglielmelli, G. Barosi, L. Pieri, E. Antonioli, A. Bosi, and A. M. Vannucchi JAK2V617F mutational status and allele burden have little influence on clinical phenotype and prognosis in patients with post-polycythemia vera and post-essential thrombocythemia myelofibrosis Haematologica, January 1, 2009; 94(1): 144 - 146. [Full Text] [PDF] J.-J. Lataillade, O. Pierre-Louis, H. C. Hasselbalch, G. Uzan, C. Jasmin, M.-C. Martyre, M.-C. Le Bousse-Kerdiles, and on behalf of the French INSERM and the European EU Does primary myelofibrosis involve a defective stem cell niche? From concept to evidence Blood, October 15, 2008; 112(8): 3026 - 3035. [Abstract] [Full Text] [PDF] R. L. Levine and D. G. Gilliland Myeloproliferative disorders Blood, September 15, 2008; 112(6): 2190 - 2198. [Abstract] [Full Text] [PDF] R. L. Levine and M. Heaney New Advances in the Pathogenesis and Therapy of Essential Thrombocythemia Hematology, January 1, 2008; 2008(1): 76 - 82. [Abstract] [Full Text] [PDF]

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