Antecedent cardiovascular disease and autoimmunity in Philadelphia-negative chronic myeloproliferative neoplasms

• We examine associations between MPNs and prior autoimmune diseases.
• We report an increased risk of MPNs with prior autoimmune disease.
• We evaluate the prediagnostic cardiovascular disease of MPNs
• We discuss the association between MPNs and chronic inflammation.

IntroductionA single institution case-control study was conducted to evaluate the risk of developing chronic myeloproliferative neoplasms (MPNs) associated with prior autoimmune disease and cardiovascular disease (CVD).MethodCases were 323 MPN patients and controls were 333 chronic lymphocytic leukemia (CLL) patients. Odds ratios (ORs) and p-values using Fischer’s exact tests were calculated. The data was adjusted for confounding effects by logistic regression.ResultsA significantly increased risk of MPNs compared to CLL was observed in subjects with a prior history of any autoimmune disease (OR = 1.86, 95%CI 1.16–2.98). A positive association between JAK2-V617F positive MPN and any autoimmune disease was observed at follow-up (OR = 2.62, 95% CI 1.21–5.67). A significantly increased risk of MPN compared to CLL was also observed in subjects with prior thromboembolic events (TE-events) (OR = 2.09, 95%CI 1.42–3.08). In the MPN group, an increased risk of JAK2-V617F-positive disease was observed in subjects with prior TE-events (OR = 2.19, 95%CI 1.51–3.16).DiscussionIt is discussed if chronic inflammation in relation to autoimmunity and atherosclerosis might elicit mutations in the hematopoietic stem cell resulting in MPNs, or whether this association actually reflects a long-lasting pre-MPN-diagnosis phase, in which the MPN-disease per se contributes to a chronic inflammatory state and immune deregulation.