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Cited by 261

The DOI matches its Crossref record; 18 of the 18 references it cites were found in a registry or an index.

Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24 577 first-degree relatives of 11 039 patients with myeloproliferative neoplasms in Sweden

Open access
Landgren, Ola, Goldin, Lynn R., Kristinsson, Sigurdur Y., Helgadottir, Elin A., Samuelsson, Jan, Björkholm, Magnus·2008·Blood

Abstract

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.

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Metadata

Authors
Landgren, O., Goldin, L. R., Kristinsson, S. Y., Helgadottir, E. A., Samuelsson, J., & Björkholm, M.
Venue
Blood
Published
2008-09-15
Volume
112
Issue
6
Pages
2199-2204
ISSN
  • 0006-4971 (ISSN-L)
  • 1528-0020
OpenAlex
W2060707701
Cited by
261
References
18
FWCI
11.49
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24 577 first-degree relatives of 11 039 patients with myeloproliferative neoplasms in Sweden. https://app.cochat.ai/papers/10.1182/blood-2008-03-143602
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Landgren, O., Goldin, L. R., Kristinsson, S. Y., Helgadottir, E. A., Samuelsson, J., & Björkholm, M. (2008). Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24 577 first-degree relatives of 11 039 patients with myeloproliferative neoplasms in Sweden. Blood. https://doi.org/10.1182/blood-2008-03-143602

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