Evaluation of vascular events in patients with myeloproliferative syndromes and mutations of either the januskinase-2 or calreticulin gene at the university hospital Krems from 2008 to 2015

Abstract

Myeloproliferative neoplasms (MPN), classified as polycythemia vera (PV), essential thrombocytosis (ET) and myelofibrosis (MF) are stem-cell derived disorders. Mutations in either the januskinase-2 (JAK-2) or the calreticulin (CALR) gene are characteristic for MPN and may result in enhanced proliferation of red blood cells, white blood cells and platelets, and thus increase the risk for vascular events.This study is a retrospective and descriptive analysis of records of patients, who underwent treatment for myeloproliferative syndromes at the Department of Hemato-Oncology of the University hospital Krems from 2008 to the end of 2015. Out of 250 patients, who were suspected for MPN, 51 patients displayed a JAK-2 V617F mutation. These were analyzed with regard to their blood values, gender, age at diagnosis, therapy and vascular events before and after diagnosis (during therapy). Of the 51 patients diagnosed with MPN and a JAK-2 V617F mutation, 33 suffered from PV, 15 from ET and 3 from MF. More men than women were diagnosed with MPN and the median age at diagnosis was 72 years. Acetylsalicylic acid, phlebotomy and Hydroxyurea were the most frequent therapies applied. In our study cohort, the most common vascular events were acute coronary syndrome and transitory ischemic attack. Thromboembolic events were effectively reduced by MPN therapy while no elevation in bleeding events could be observed.

A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms

“…A full-text review of the remaining 117 articles was undertaken and 88 articles were found to be ineligible for the meta-analysis (16 articles were reviews, meta-analysis or commentaries; 3 articles did not recruit patients with MPNs; and 69 articles did not report our outcome of interest). Finally, 29 cohort studies (8 prospective studies and 21 retrospective studies) were included in the meta-analysis [7–35]. The literature review and identification process are summarized as Fig.…”

“…At diagnosis, the pooled prevalence of overall thrombosis (either arterial or venous) among patients with MPN was 20.0% (95% CI, 16.6–23.8%; I 2 96%; Fig. 2) [7–18, 20–23, 25–32, 34, 35]. The pooled prevalence for each MPN subtype was as followed; PV 28.6% (95% CI, 22.0–36.3%; I 2 95%) [10, 12, 14, 18, 19, 26, 28, 29, 31, 32], ET 20.7% (95% CI, 16.6–25.5%; I 2 93%) [7–11, 13, 16–18, 22, 25, 26, 28, 29, 31, 32, 34], and PMF 9.5% (95% CI, 5.0–17.4%; I 2 94%) [10, 20, 21, 23, 26, 28, 29, 32] (Fig.…”

“…The pooled prevalence of arterial thrombosis was 16.2% (95% CI, 13.0–20.0%; I 2 95%) [7–14, 17–23, 26–29, 31, 32, 34, 35] while the pooled prevalence of venous thrombosis was 6.2% (95% CI, 4.9–7.8%; I 2 89%) (Fig. 4) [7–14, 17–23, 26, 28, 29, 31, 32, 34, 35].

Fig.

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“…Similarly, bleeding in these patients can be a minor one or could manifest as major internal organ hemorrhage [6]. The reported prevalence of thrombosis and bleeding among patients who were newly diagnosed with MPN varied considerably across the studies [7–35]. As one of the aims of the treatments of MPN is to decrease the risk of thrombosis and some prescribed medications, such as aspirin, can increase the risk of bleeding, knowing the baseline prevalence of thrombosis and bleeding would be of clinical importance for clinicians who need to balance the risk between these two opposite complications.…”

Molecular Links between Erythrocyte Adhesion and Vascular Dysfunction in Diabetes Mellitus, Polycythemia Vera, Retinal Vascular Occlusion

“…In addition the use of anti-platelet agents can increase the risk of hemorrhage. The most common vascular events were acute coronary syndrome and transitory ischemic attack [18]. Because adhesion of RBCs to endothelium was shown to be correlated to vascular risk in sickle cell anemia and diabetes mellitus we investigated whether RBCs from patients with PV had abnormal interactions with endothelium by measuring RBC adhesion to Human Umbilical Vein Endothelial Cells (HUVEC) under static conditions (Figure 3) [19].…”