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Blood, 1 December 2007, Vol. 110, No. 12, pp. 4073-4076. Prepublished online as a Blood First Edition Paper on August 21, 2007; DOI 10.1182/blood-2007-06-095554. This Article Full Text (PDF) Supplemental Figures All Versions of this Article: blood-2007-06-095554v1 110/12/4073    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 Catalano, A. Articles by Iland, H. Search for Related Content PubMed PubMed Citation Articles by Catalano, A. Articles by Iland, H. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted June 28, 2007 Accepted August 14, 2007 The PRKAR1A gene is fused to RARA in a new variant acute promyelocytic leukemia Alberto Catalano*, Mark A Dawson, Karthiga Somana, Stephen Opat, Anthony Schwarer, Lynda J Campbell, and Harry Iland Institute of Haematology, Royal Prince Alfred Hospital, Sydney, NSW, Australia Clinical Haematology and Bone Marrow Transplant Unit, The Alfred Hospital, Melbourne, Victoria, Australia Victorian Cancer Cytogenetics Service, St Vincent's Hospital, Melbourne, Victoria, Australia * Corresponding author; email: alberto.catalano{at}email.cs.nsw.gov.au. We report the molecular and cytogenetic characterization of a novel variant of acute promyelocytic leukemia (APL). The bone marrow showed 88% hypergranular promyelocytes and the karyotype was 47,XY,+22[5]/46,XY[30]. FISH indicated disruption and deletion of the 5'-end of the RARA gene. Treatment with ATRA, idarubicin and arsenic trioxide induced cytogenetic complete remission without morphological evidence of residual leukemia. The diagnostic marrow was negative for PML-RARA transcripts by RT-PCR, but an atypical product was observed. Sequencing showed partial homology to the PRKAR1A gene, encoding the regulatory subunit type I-alpha of cyclic AMP-dependent protein kinase. RT-PCR using PRKAR1A and RARA specific primers amplified two transcript splice variants of a PRKAR1A-RARA fusion gene. This novel PRKAR1A-RARA gene rearrangement is the fifth variant APL in which the RARA partner gene has been identified and the second known rearrangement of PRKAR1A in a malignant disease. This trial has been registered at www.actr.org.au/ with the Australian Clinical Trials Registry, registration #12605000070639. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: C. F. Burande, M. L. Heuze, I. Lamsoul, B. Monsarrat, S. Uttenweiler-Joseph, and P. G. Lutz A Label-free Quantitative Proteomics Strategy to Identify E3 Ubiquitin Ligase Substrates Targeted to Proteasome Degradation Mol. Cell. Proteomics, July 1, 2009; 8(7): 1719 - 1727. [Abstract] [Full Text] [PDF] M. A. Sanz, D. Grimwade, M. S. Tallman, B. Lowenberg, P. Fenaux, E. H. Estey, T. Naoe, E. Lengfelder, T. Buchner, H. Dohner, et al. Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet Blood, February 26, 2009; 113(9): 1875 - 1891. [Abstract] [Full Text] [PDF] T. Kondo, A. Mori, S. Darmanin, S. Hashino, J. Tanaka, and M. Asaka The seventh pathogenic fusion gene FIP1L1-RARA was isolated from a t(4;17)-positive acute promyelocytic leukemia Haematologica, September 1, 2008; 93(9): 1414 - 1416. [Full Text] [PDF]

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