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Blood, 1 December 2008, Vol. 112, No. 12, pp. 4694-4698. Prepublished online as a Blood First Edition Paper on September 12, 2008; DOI 10.1182/blood-2008-02-136382. This Article Full Text (PDF) Supplemental Figures All Versions of this Article: blood-2008-02-136382v1 112/12/4694    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 Epling-Burnette, P.K. Articles by Wei, S. Search for Related Content PubMed PubMed Citation Articles by Epling-Burnette, P.K. Articles by Wei, S. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted February 1, 2008 Accepted June 10, 2008 Clinical improvement by farnesyltransferase inhibition in NK large granular lymphocyte leukemia associated with imbalanced NK receptor signaling P.K. Epling-Burnette*, Lubomir Sokol, Xianhong Chen, Fanqi Bai, Junmin Zhou, Michelle A. Blaskovich, JianXiang Zou, Jeffrey S. Painter, Todd D. Edwards, Lynn Moscinski, Jeffrey A. Yoder, Julie Y. Djeu, Said Sebti, Thomas P Loughran Jr, and Sheng Wei H. Lee Moffitt Cancer Center and Research Institute Immunology Program, Department of Interdisciplinary Oncology, University of South Florida, Tampa, FL, United States H. Lee Moffitt Cancer Center and Research Institute, Malignant Hematology Division, Department of Interdisciplinary Oncology, University of South Florida, Tampa, FL, United States The Stern Cardiovascular Center, Germantown, TN, United States H. Lee Moffitt Cancer Center and Research Institute, Pathology Division, Department of Interdisciplinary Oncology, University of South Florida, Tampa, FL, United States Department of Molecular Biomedical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh, NC, United States Penn State Cancer Institute, Penn State College of Medicine, Hershey, PA, United States * Corresponding author; email: pearlie.burnette{at}moffitt.org. Large Granular lymphocyte (LGL) leukemia is commonly associated with poor hematopoiesis. The first case of pulmonary artery hypertension (PAH) was observed in a 57-year-old woman with NK-LGL leukemia and transfusion-dependent anemia. Using a genetic approach, we demonstrated that killing of pulmonary endothelial cells by patient NK-cells was mediated by dysregulated balance in activating and inhibitory NK-receptor signaling. Elevated pulmonary artery pressure and erythroid differentiation improved after disrupting the NK-receptor signaling pathway with four courses of a farnesyltransferase inhibitor tipifarnib. Coincidental association between PAH and LGL leukemia suggest a causal relationship between the expanded lymphocyte population and these clinical manifestations. This trial has been registered at www.ClinicalTrials.gov, NCI 6823 CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: R. Zambello and G. Semenzato Large granular lymphocyte disorders: new etiopathogenetic clues as a rationale for innovative therapeutic approaches Haematologica, October 1, 2009; 94(10): 1341 - 1345. [Full Text] [PDF]

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