Abstract
The JAK2V617F mutation is an acquired somatic mutation, which is prevalent in patients with the Philadelphia-chromosome negative myeloproliferative neoplasms (MPNs). In these diseases the mutation gives rise to constitutive JAK-STAT signaling with increased blood cell counts and in vivo activation of neutrophils and platelets as well, which altogether contribute to a chronic inflammatory and thrombogenic state with a 12-fold increased risk of coronary disease. Treatment with recombinant interferon-alpha2 (rIFN) reduces the JAK2V617F allelic burden in a large number of MPN-patients. Long-term treatment with rIFN associates with low-burden JAK2V617F in a subset of patients and a decreased thrombosis risk as well. In the general population the JAK2V617F mutation has been shown to associate with ischemic heart disease and thrombosis. Based upon the above observations we herein report the first patient with CHIP-JAK2V617F, in whom treatment with rIFN resolved severe angina pectoris. During a short period off rIFN the symptoms reappeared to resolve in concert with reduction of JAK2V617F allele burden, when rIFN was reinstituted. The JAK2V617F mutation may be a novel therapeutic target to prohibit the development of cardiovascular diseases using rIFN either as monotherapy or in combination with potent anti-inflammatory agents.
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