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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 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 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 Landgren, O. Articles by Björkholm, M. Search for Related Content PubMed PubMed Citation Articles by Landgren, O. Articles by Björkholm, M. Related Collections Neoplasia Plenary Papers Free Research Articles Clinical Trials and Observations Related Articles in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  PLENARY PAPER Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24 577 first-degree relatives of 11 039 patients with myeloproliferative neoplasms in Sweden Ola Landgren1,2, Lynn R. Goldin1, Sigurdur Y. Kristinsson2, Elin A. Helgadottir2, Jan Samuelsson3,4, and Magnus Björkholm2,4 1 National Cancer Institute, National Institutes of Health, Bethesda, MD; 2 Department of Medicine, Division of Hematology, Karolinska University Hospital and Institutet, Stockholm, Sweden; 3 Department of Medicine, Stockholm South Hospital, Stockholm, Sweden; and 4 Swedish Myeloproliferative Disorder Study Group, Stockholm, Sweden Previous small studies have reported familial clustering of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). We identified 6217 PV, 2838 ET, 1172 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 (RRs) 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, which can be confirmed only by identifying the susceptibility gene(s). Mean age at MPN diagnosis was not different (P = .20) 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 = .10), 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 = .09). Our findings of 5- to 7-fold elevated risk of MPNs among first-degree relatives of MPN patients support the hypothesis that common, strong, shared susceptibility genes predispose 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. 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