Rinsho Kensa Volume 54, Issue 3 (March 2010)

Treatment―guideline, outcome, prognosis:primary myelofibrosis Jo Ishizawa 1 , Shinichiro Okamoto 1 1Division of Hematology, Department of Internal Medicine, Keio University School of Medicine Keyword: reduced-intensity conditioning , JAK/STAT pathway , molecular targeting agents pp.293-298
Published Date 2010/3/15
DOI https://doi.org/10.11477/mf.1542102256
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Primary myelofibrosis is a clonal myeloproliferative neoplasm characterized by anemia, marked splenomegaly, and bone marrow fibrosis. Median survival time is estimated at 3-5 years, and causes of deaths include leukemic transformation and consequences of progressive cytopenia. Treatment remains largely palliative with the exception of allogeneic stem cell transplantation, which is the only curative approach in some patients. However, the identification of the JAK2 V617F mutation in more than half of the patients may improve our understanding of the disease biology, and clinical phase Ⅰ/Ⅱ trials of the drugs targeting this mutation or the associated molecular abnormalities are currently underway. In this review we will update the current therapeutic options as well as the development of innovative molecular targeting agents for primary myelofibrosis.

Copyright © 2010, Igaku-Shoin Ltd. All rights reserved.


54巻3号 (2010年3月)
電子版ISSN 1882-1367 印刷版ISSN 0485-1420 医学書院