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Blood. 2008 Dec 1;112(12):4694-8. doi: 10.1182/blood-2008-02-136382. Epub 2008 Sep 12.

Clinical improvement by farnesyltransferase inhibition in NK large granular lymphocyte leukemia associated with imbalanced NK receptor signaling.

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  • 1Department of Interdisciplinary Oncology, H Lee Moffitt Cancer Center and Research Institute Immunology Program, James A Haley Veterans Administration Hospital, University of South Florida, Tampa, FL 33612, USA. Pearlie.Burnette@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 natural killer (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 4 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 is registered at www.ClinicalTrials.gov as NCI 6823.

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