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Blood, 15 September 2008, Vol. 112, No. 6, pp. 2199-2204. Prepublished online as a Blood First Edition Paper on May 1, 2008; DOI 10.1182/blood-2008-03-143602. This Article Full Text (PDF) All Versions of this Article: blood-2008-03-143602v1 112/6/2199    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 Landgren, O. Articles by Bjorkholm, M. Search for Related Content PubMed PubMed Citation Articles by Landgren, O. Articles by Bjorkholm, M. Related Collections Related Articles in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted March 7, 2008 Accepted April 12, 2008 Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24577 first-degree relatives of 11039 patients with myeloproliferative neoplasms in Sweden Ola Landgren*, Lynn R. Goldin, Sigurdur Y Kristinsson, Elin A Helgadottir, Jan Samuelsson, and Magnus Bjorkholm National Cancer Institute, National Institutes of Health, Bethesda, MD, United States Department of Medicine, Division of Hematology, Karolinska University Hospital and Institutet, Stockholm, Sweden Department of Medicine, Stockholm South Hospital, Stockholm, Sweden Swedish Myeloproliferative Disorder Study Group, Stockholm, Sweden * Corresponding author; email: landgreo{at}mail.nih.gov. Previous small studies have reported familial clustering of myeloproliferative neoplasms (MPN) including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). We identified 6,217 PV, 2,838 ET, 1,172 MF, and 812 MPN unclassifiable (NOS) patients diagnosed in Sweden, 43,550 controls, and first-degree relatives of cases (n=24,577) and controls (n=99,542). Using a marginal survival model we calculated relative risks (RR) and 95% confidence intervals as measures of familial aggregation. Relatives of MPN patients had significantly increased risks of PV (RR=5.7; 3.5-9.1), ET (RR=7.4; 3.7-14.8), and MPN NOS (RR=7.5; 2.7-20.8). Analyses stratified by type of first-degree relative revealed consistently higher risks for siblings compatible with a model of recessive genetic inheritance, although this can only be confirmed by identifying the actual susceptibility gene(s). Mean age at MPN diagnosis was not different (p=0.20, t-test) for affected relatives of cases (57.5 years) versus controls (60.6 years) and risk of MPN by age was not different for parents versus offspring of MPN cases (p=0.10, log-rank test) providing no support for anticipation. Relatives of MPN patients had a borderline increased risk of chronic myeloid leukemia (CML) (RR=1.9; 0.9-3.8; p=0.09). Our findings of 5- to 7-fold elevated risk of MPNs among first-degree relatives of MPN patients support the hypothesis that there are common, strong, shared susceptibility genes predisposing to PV, ET, MF and possibly CML. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Articles in Blood Online: MPDs: it's all in the family Jerry L. Spivak Blood 2008 112: 2173-2174. [Full Text] [PDF] Disease anticipation in familial myeloproliferative neoplasms Elisa Rumi, Francesco Passamonti, Cristina Picone, Matteo Giovanni Della Porta, Cristiana Pascutto, Mario Cazzola, and Mario Lazzarino Blood 2008 112: 2587-2588. [Full Text] [PDF] Response: More on disease anticipation in familial MPN Ola Landgren, Lynn R. Goldin, Sigurdur Y. Kristinsson, Elin A. Helgadottir, Jan Samuelsson, and Magnus Bjorkholm Blood 2008 112: 2588-2589. [Full Text] [PDF] This article has been cited by other articles: A. M. Vannucchi, G. Masala, E. Antonioli, M. Chiara Susini, P. Guglielmelli, L. Pieri, L. Maggi, S. Caini, D. Palli, C. Bogani, et al. Increased Risk of Lymphoid Neoplasms in Patients with Philadelphia Chromosome-Negative Myeloproliferative Neoplasms Cancer Epidemiol. Biomarkers Prev., July 1, 2009; 18(7): 2068 - 2073. [Abstract] [Full Text] [PDF] L. R. Goldin, M. Bjorkholm, S. Y. Kristinsson, I. Turesson, and O. Landgren Elevated risk of chronic lymphocytic leukemia and other indolent non-Hodgkin's lymphomas among relatives of patients with chronic lymphocytic leukemia Haematologica, May 1, 2009; 94(5): 647 - 653. [Abstract] [Full Text] [PDF] A. M. Vannucchi, P. Guglielmelli, and A. Tefferi Advances in Understanding and Management of Myeloproliferative Neoplasms CA Cancer J Clin, May 1, 2009; 59(3): 171 - 191. [Abstract] [Full Text] [PDF] Familial Myeloproliferative Neoplasms Journal Watch Oncology and Hematology, October 7, 2008; 2008(1007): 3 - 3. [Full Text] E. Rumi, F. Passamonti, C. Picone, M. G. Della Porta, C. Pascutto, M. Cazzola, and M. Lazzarino Disease anticipation in familial myeloproliferative neoplasms Blood, September 15, 2008; 112(6): 2587 - 2588. [Full Text] [PDF] O. Landgren, L. R. Goldin, S. Y. Kristinsson, E. A. Helgadottir, J. Samuelsson, and M. Bjorkholm Response: More on disease anticipation in familial MPN Blood, September 15, 2008; 112(6): 2588 - 2589. 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