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Blood, 1 September 2006, Vol. 108, No. 5, pp. 1497-1503. Prepublished online as a Blood First Edition Paper on May 4, 2006; DOI 10.1182/blood-2006-03-009746. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2006-03-009746v1 108/5/1497    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 Tefferi, A. Articles by Kantarjian, H. Search for Related Content PubMed PubMed Citation Articles by Tefferi, A. Articles by Kantarjian, H. Related Collections Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS International Working Group (IWG) consensus criteria for treatment response in myelofibrosis with myeloid metaplasia, for the IWG for Myelofibrosis Research and Treatment (IWG-MRT) Ayalew Tefferi, Giovanni Barosi, Ruben A. Mesa, Francisco Cervantes, H. Joachim Deeg, John T. Reilly, Srdan Verstovsek, Brigitte Dupriez, Richard T. Silver, Olatoyosi Odenike, Jorge Cortes, Martha Wadleigh, Lawrence A. Solberg, Jr, John K. Camoriano, Heinz Gisslinger, Pierre Noel, Juergen Thiele, James W. Vardiman, Ronald Hoffman, Nicholas C. P. Cross, D. Gary Gilliland, and Hagop Kantarjian From the Mayo Clinic, Rochester, MN; Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; Hospital Clinic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; the Fred Hutchinson Cancer Research Center, Seattle, WA; the Royal Hallamshire Hospital, Sheffield, United Kingdom; the M. D. Anderson Cancer Center, Houston, TX; Service d'Hématologie Clinique, Centre Hospitalier de Lens, France; Cornell Medical Center, New York, NY; the University of Chicago, IL; the Dana Farber Cancer Institute, Boston, MA; the Mayo Clinic, Jacksonville, FL; the Mayo Clinic, Scottsdale, AZ; the Department of Hematology and Blood Coagulation, Medical University of Vienna, Austria; the National Institutes of Health, Bethesda, MD; the Institute of Pathology, University of Cologne, Germany; the University of Illinois, Chicago and the Wessex Regional Genetics Laboratory, Salisbury, United Kingdom. Myelofibrosis with myeloid metaplasia (MMM) is a clinicopathologic entity characterized by stem cell-derived clonal myeloproliferation, ineffective erythropoiesis, extramedullary hematopoiesis, and bone marrow fibrosis and osteosclerosis. Patients with MMM have shortened survival and their quality of life is compromised by progressive anemia, marked hepatosplenomegaly, and severe constitutional symptoms including cachexia. After decades of frustration with ineffective therapy, patients are now being served by promising treatment approaches that include allogeneic hematopoietic stem cell transplantation and immunomodulatory drugs. Recent information regarding disease pathogenesis, including a contribution to the myeloproliferative disorder phenotype by a gain-of-function JAK2 mutation (JAK2V617F), has revived the prospect of targeted therapeutics as well as molecular monitoring of treatment response. Such progress calls for standardization of response criteria to accurately assess the value of new treatment modalities, to allow accurate comparison between studies, and to ensure that the definition of response reflects meaningful health outcome. Accordingly, an international panel of experts recently convened and delineated 3 response categories: complete remission (CR), partial remission (PR), and clinical improvement (CI). Bone marrow histologic and hematologic remissions characterize CR and CR/PR, respectively. The panel agreed that the CI response category is applicable only to patients with moderate to severe cytopenia or splenomegaly. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: R. A. Mesa How I treat symptomatic splenomegaly in patients with myelofibrosis Blood, May 28, 2009; 113(22): 5394 - 5400. [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] N. Kroger, H. Alchalby, E. Klyuchnikov, A. Badbaran, Y. Hildebrandt, F. Ayuk, U. Bacher, O. Bock, M. Kvasnicka, B. Fehse, et al. JAK2-V617F-triggered preemptive and salvage adoptive immunotherapy with donor-lymphocyte infusion in patients with myelofibrosis after allogeneic stem cell transplantation Blood, February 19, 2009; 113(8): 1866 - 1868. [Full Text] [PDF] A. D. Pardanani, J. Gotlib, C. Jamieson, J. Cortes, M. Talpaz, R. M. Stone, M. H Silverman, J. Shorr, D. G. Gilliland, and A. Tefferi A Phase I Study of TG101348, An Orally Bioavailable JAK2-Selective Inhibitor, in Patients with Myelofibrosis Blood (ASH Annual Meeting Abstracts), November 16, 2008; 112(11): 97 - 97. [Abstract] A. Tefferi, S. Verstovsek, G. Barosi, F. Passamonti, G. J. Roboz, H. Gisslinger, R. Paquette, F. Cervantes, C. E. Rivera, H. J. Deeg, et al. Pomalidomide Therapy in Anemic Patients with Myelofibrosis: Results from a Phase-2 Randomized Multicenter Study Blood (ASH Annual Meeting Abstracts), November 16, 2008; 112(11): 663 - 663. [Abstract] R. Weinkove, J. T. Reilly, M. F. McMullin, N. J. Curtin, D. Radia, and C. N. Harrison Low-dose thalidomide in myelofibrosis Haematologica, July 1, 2008; 93(7): 1100 - 1101. [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] D. A. Rizzieri, E. Feldman, J. F. DiPersio, N. Gabrail, W. Stock, R. Strair, V. M. Rivera, M. Albitar, C. L. Bedrosian, and F. J. Giles A Phase 2 Clinical Trial of Deforolimus (AP23573, MK-8669), a Novel Mammalian Target of Rapamycin Inhibitor, in Patients with Relapsed or Refractory Hematologic Malignancies Clin. Cancer Res., May 1, 2008; 14(9): 2756 - 2762. [Abstract] [Full Text] [PDF] N. Kroger, A. Badbaran, E. Holler, J. Hahn, G. Kobbe, M. Bornhauser, A. Reiter, T. Zabelina, A. R. Zander, and B. Fehse Monitoring of the JAK2-V617F mutation by highly sensitive quantitative real-time PCR after allogeneic stem cell transplantation in patients with myelofibrosis Blood, February 1, 2007; 109(3): 1316 - 1321. [Abstract] [Full Text] [PDF] R. Hoffman and D. Rondelli Biology and Treatment of Primary Myelofibrosis Hematology, January 1, 2007; 2007(1): 346 - 354. [Abstract] [Full Text] [PDF]

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