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Autoimmunity and the risk of myeloproliferative neoplasms

¹ Department of Medicine, Division of Hematology and Hematological Laboratory, Centrum for Molecular Medicine, Karolinska University Hospital Solna and Karolinska Institutet, Stockholm, Sweden
² Swedish Myeloproliferative Disorder Study Group, Stockholm, Sweden
³ Medical Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
⁴ Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
⁵ Department of Clinical Science and Education Karolinska Institutet, Department of Internal Medicine, Stockholm South Hospital, Stockholm, Sweden

Correspondence: Sigurdur Yngvi Kristinsson M.D., Ph.D., Department of Medicine, Division of Hematology, Karolinska University Hospital Solna, SE-171 76 Stockholm, Sweden. Phone: international +46.8.51770000. Fax: international+46.8.318264. E-mail: sigurdur.kristinsson{at}karolinska.se

ABSTRACT

The causes of myeloproliferative neoplasm (MPN) are unknown. We conducted a large population-based study including 11,039 MPN patients and 43,550 matched controls with the aim to assess the associations between a personal history of a broad span of autoimmune diseases and subsequent risk of MPN. We found a prior history of any autoimmune disease to be associated with a significantly increased risk of MPNs (odds ratio (OR)=1.2; 95% confidence interval (CI) 1.0–1.3; p= 0.021). Specifically, we found an increased risk of MPNs associated with a prior immune thrombocytopenic purpura (2.9; 1.7–7.2), Crohn’s disease (1.8; 1.1–3.0), polymyalgia rheumatica (1.7; 1.2–2.5), giant cell arteritis (5.9; 2.4–14.4), Reiter’s syndrome (15.9; 1.8–142) and aplastic anemia (7.8; 3.7–16.7). The risk of MPNs associated with prior autoimmune diseases is modest but statistically significant. Future studies are needed to unravel the effects of these autoimmune diseases themselves, their treatment, or common genetic susceptibility.

Key words: myeloproliferative disorders, autoimmune diseases, polycythemia vera, essential thrombocythemia, primary myelofibrosis, Crohn’s disease, immune thrombocytopenic purpura, giant cell arteritis, polymyalgia rheumatica, aplastic anemia.

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