Journal de la leucémie
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Abstrait

Resolution of Angina Pectoris in Five Patients with Myeloproliferative Neoplasms Treated with Pegylated Interferon Resistant Anginal: MPN Patient Treated with PEG-IFN

Miklos Egyed*, Eszter Kovacs, Eva Karadi, Jozsef Herczeg, Béla Kajtár, Lasse Kjaer, Vibe Skov, Hans Carl Hasselbalch

The Philadelphia-negative chronic Myeloproliferative Neoplasms (MPNs) are characterised by the prevalence of somatic mutation JAK2V617F and associated with a high Cardiovascular Disease (CVD) burden, including ischemic heart disease with angina pectoris and heart failure, peripheral arterial insufficiency and risk the increase of such vascular malformations as aneurisms. Firing of the mutant Janus Kinase/Signal Transducers And Activators Of Transcription (JAK-STAT) signal transduction pathway results in blood cell count increase and induces the differentiation and maturation process of platelets and neutrophils. All these concomitant processes enhance the establishment of chronic inflammatory and thrombogenic state with a 12-times higher risk of coronary disease as a consequence. Long-term administration of Interferon-Alpha2 (rIFN) is proven to reduce the JAK2V617F allelic burden and minimise the risk of thrombotic events. Our first case report has already been published about a CHIP-JAK2V617F patient, who had gained remarkable relief of his treatment resistant angina pectoris due to a low dose of rIFN. Our present report describes five angina pectoris patient cases who had been diagnosed with MPN as well and showed significant improvement of their cardiological disease, refractor to previous therapy, instantly after the start of treatment with rIFN. The pathophysiological mechanisms behind such a remarkable rIFN-induced anti-angina pectoris effect is discussed hereafter. Our previous report and this series of patients call for the launch of explorative investigation of the rIFN effect on patients with CVD and MPN comorbidities or with CHIP-JAK2V617F disease predisposition.

Key points: 1. Complete resolution of angina pectoris is reported in five JAK2V617F-positive MPN-patients with severe ischemic heart disease during treatment with rIFN. 2. Targeting the JAK2V617F mutation by rIFN may favourably impact the CVD disease burden in MPNs to be pursued in future trials.