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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 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 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 Catalano, A. Articles by Iland, H. Search for Related Content PubMed PubMed Citation Articles by Catalano, A. Articles by Iland, H. Related Collections Neoplasia Oncogenes and Tumor Suppressors Brief Reports Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  NEOPLASIA Brief Report The PRKAR1A gene is fused to RARA in a new variant acute promyelocytic leukemia Alberto Catalano1, Mark A. Dawson2, Karthiga Somana3, Stephen Opat2, Anthony Schwarer2, Lynda J. Campbell3, and Harry Iland1 1 Institute of Haematology, Royal Prince Alfred Hospital, Sydney; 2 Clinical Haematology and Bone Marrow Transplant Unit, the Alfred Hospital, Melbourne; and 3 Victorian Cancer Cytogenetics Service, St Vincent's Hospital, Melbourne, Australia 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]. Fluorescence in situ hybridization (FISH) indicated disruption and deletion of the 5'-end of the RARA gene. Treatment with all-trans retinoic acid, idarubicin, and arsenic trioxide induced cytogenetic complete remission without morphologic evidence of residual leukemia. The diagnostic marrow was negative for PML-RARA transcripts by reverse transcription–polymerase chain reaction (RT-PCR), but an atypical product was observed. Sequencing showed partial homology to the PRKAR1A gene, encoding the regulatory subunit type I- of cyclic adenosine monophosphate–dependent protein kinase. RT-PCR using specific primers for PRKAR1A and RARA amplified 2 transcript splice variants of a PRKAR1A-RARA fusion gene, and PRKAR1A and RARA FISH probes confirmed the fusion. 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 was registered at www.actr.org.au with the Australian Clinical Trials Registry as number 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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