Coronary microvascular dysfunction due to essential thrombocythemia and policythemia vera: the missing piece in the puzzle of their increased cardiovascular risk?

Myeloproliferative neoplasms are most commonly associated with venous thrombosis. Up to 60% of patients experience a thrombotic event in their lifetimes, including stroke or myocardial infarction. It is unclear whether pathogenetic factors linking essential thrombocythemia (ET) and polycythemia vera (PV) to thrombotic complications do play a role in the risk of coronary artery disease (CAD).

Coagulation activation in children with sickle cell disease is associated with cerebral small vessel vasculopathy.

Background: Thrombotic complications in Sickle Cell Disease (SCD) arise since infancy, but the role of the coagulation system in children has been poorly explored. To determine its role in the development of clinical complications in childhood we measured coagulation and endothelial parameters in children with SCD at steady state.

Methods: Markers of thrombin generation, fibrin dissolution and endothelial activation were evaluated in 38 children with SS-Sβ°, 6 with SC disease and 50 age and blood group matched controls.

Circulating microparticles of glial origin and tissue factor bearing in high-grade glioma: a potential prothrombotic role.

Venous thromboembolism (VTE) may complicate the clinical course of glioblastoma multiforme (GBM). Circulating microparticles (MPs) have been associated with cancer-related VTE. Sixty-one consecutive patients with GBM undergoing gross-total (41) or subtotal (20) surgical resection followed by radio-chemotherapy were prospectively evaluated.

Contribution of fibrinolytic tests to the differential diagnosis of veno-occlusive disease complicating pediatric hematopoietic stem cell transplantation.

Background: Veno-occlusive disease (VOD) is a major complication following hematopoietic stem cell transplantation (HSCT). Its diagnosis is based on clinical criteria, which have a limited sensitivity. Increased plasminogen activator inhibitor-1 (PAI-1) levels have been suggested as a marker of VOD.

Defining the thrombotic risk in patients with myeloproliferative neoplasms.

Polycythemia vera (PV) and essential thrombocythemia (ET) are two Philadelphia-negative myeloproliferative neoplasms (MPN) associated with an acquired mutation in the JAK2 tyrosine kinase gene. There is a rare incidence of progression to myelofibrosis and myeloid metaplasia in both disorders, which may or may not precede transformation to acute myeloid leukemia, but thrombosis is the main cause of morbidity and mortality. The pathophysiology of thrombosis in patients with MPN is complex.

von Willebrand factor abnormalities in aortic valve stenosis: Pathophysiology and impact on bleeding.

Acquired von Willebrand syndrome (AVWS) may complicate severe aortic valve stenosis, due to a reduction in the haemostatically more efficient large von Willebrand factor (VWF) multimers. This study was designed to analyse the relevance of VWF abnormalities and haemorrhagic diathesis in severe aortic valve stenosis. Forty-one consecutive patients undergoing valve replacement were investigated: seven had minor bleeding symptoms in their recent history; 10 (24.

Prothrombotic state in glioblastoma multiforme: an evaluation of the procoagulant activity of circulating microparticles.

The relationship between venous thromboembolism (VTE) and cancer is supported by several pathogenetic factors, including circulating microparticles (MP) originating from different cells and often bearing tissue factor. Since VTE often complicates the clinical course of patients with glioblastoma multiforme (GBM; WHO grade IV astrocytoma) and the role of MPs in these patients population is still not clear, this prospective study was conducted to evaluate the procoagulant activity of circulating MP (MP activity) in GBM patients. We enrolled 61 GBM patients undergoing gross-total or subtotal surgical resection followed by combined radio-chemotherapy; 20 healthy volunteers were tested as controls.

Nitric oxide derivatives and soluble plasma selectins in patients with myeloproliferative neoplasms.

Essential thrombocythaemia (ET) and polycythaemia vera (PV) are characterised by a high incidence of thrombotic complications due to high-shear stress of the vessel wall, blood hyperviscosity and hypoxaemia, all factors responsible for chronic endothelial dysfunction and platelet and leukocyte activation. We evaluated the activation status of vascular cells in 18 consecutive ET and 14 PV patients by measuring the plasma levels of the nitric oxide derivatives (NOX) (i.e.

Effects of tadalafil on platelets and endothelium in patients with erectile dysfunction and cardiovascular risk factors: a pilot study.

Activation of endothelial cells and platelets is an initial step toward the development of cardiovascular disease. Erectile dysfunction (ED) may be an early manifestation of endotheliopathy. We evaluated the effects of tadalafil on cyclic nucleotides (cGMP and cAMP) and soluble adhesion molecules (E- and P-selectin [ES and PS]).

Effect of recombinant activated factor VII in critical bleeding: clinical experience of a single center.

Recombinant activated factor VII (rFVIIa) has been successfully used ''off-label'' in patients with refractory life-threatening hemorrhage. Intravenous rFVIIa was given to 31 patients unresponsive to standard therapy with blood products and surgical reexploration, who were bleeding due to trauma, surgery, organ transplantation, liver cirrhosis, ruptured uterus. We recorded their coagulation and hematologic profiles, acid-base balance, blood loss, number of red blood cells (RBC), plasma and platelet transfusions, complications, and survival.

Effect of bosentan on plasma markers of endothelial cell activity in patients with secondary pulmonary hypertension related to connective tissue diseases.

Objective: To evaluate plasma markers of endothelial cell activity in patients with pulmonary arterial hypertension (PAH) induced by connective tissue diseases (CTD) before and after 3-month administration of bosentan.

Methods: We quantified E, L and P-selectin (sE-S, sL-S, sP-S), thrombomodulin (TM), monocyte-chemotactic protein 1 (MCP-1), human soluble CD40 ligand (sCD40L), and nitric oxide (NO) in 18 patients and 18 controls. We evaluated right ventricular systolic pressure (RVSP) and the 6-minute walk test (6-MWT).

Hyperthyroidism as a cause of pulmonary arterial hypertension: a prospective study.

The authors assessed the prevalence of pulmonary arterial hypertension (PAH) in patients with hyperthyroidism and evaluated the response to treatment of the thyrotoxicosis. They assessed the pulmonary artery systolic pressure (PASP) at rest (estimated by echocardiography) in 23 consecutive patients diagnosed with hyperthyroidism due to Graves' disease or toxic multinodular goiter. Twelve of 23 patients (52%) did not show antithyroglobulin and antithyroperoxidase antibodies.

Endothelial cell activity in chronic obstructive pulmonary disease without severe pulmonary hypertension.

Pulmonary hypertension is common in patients with chronic obstructive pulmonary disease (COPD), but the precise mechanism of vascular impairment in these patients is unknown. We, therefore, decided to investigate whether endothelial cell dysfunction is present in patients with COPD with a wide range of chronic airflow obstruction before the development of severe pulmonary hypertension. Selected plasma markers of endothelial cell activity were studied: nitrate+nitrite (NO-(2)/NO-(3)), thrombomodulin (TM), tissue factor pathway inhibitor (TFPI), soluble selectins (endothelium sES, leukocyte sLS, platelet sPS), soluble intercellular adhesion molecule-1 (sICAM-1), and soluble platelet endothelial cell adhesion molecule-1 (sPECAM-1).

Role of fibrinolytic and clotting parameters in the diagnosis of liver veno-occlusive disease after hematopoietic stem cell transplantation in a pediatric population.

Hepatic veno-occlusive disease (VOD) is a severe complication after hematopoietic stem cell transplantation (HSCT). Recent studies, mainly in adults receiving HSCT, have identified an increase in the plasminogen activator inhibitor-1 (PAI-1) as a possible marker of VOD. To confirm this finding, the fibrinolytic, coagulation and liver function parameters were assayed before and weekly for 1 month after 61 HSCT performed in 53 consecutive children.

Spontaneous evolution of essential thrombocythaemia into acute megakaryoblastic leukaemia with trisomy 8, trisomy 21 and cutaneous involvement.

Spontaneous transformation of essential thrombocythaemia (ET) into acute leukaemia is rare. We describe a case of ET that spontaneously transformed after 19 yrs uneventful follow-up into acute megakaryoblastic leukaemia. Cytogenetic analysis of bone marrow nucleated cells showed trisomy 8 and trisomy 21 at time of leukaemic transformation supporting the hypothesis that chromosomal abnormalities are part of the mechanism that drives the leukaemic progression independently of drug cytotoxicity.

Markers of endothelial function in pediatric stem cell transplantation for acute leukemia.

Background: Endothelial cells and leukocytes intimately interact in inflammation and coagulation processes, so that dysregulation of their function may lead to both cellular damage and thrombosis, which may occur as complications of bone marrow transplantation (BMT). Partially conflicting evidence about endothelial markers and their relationships with clinical complications after BMT has been reported in the literature. Since almost all studies were carried out in adults, we evaluated some recent available markers of endothelial cell function in pediatric patients undergoing stem cell transplantation (SCT) for acute leukemia.

Splenectomy after portal thrombosis in patients with polycythemia vera and essential thrombocythemia.

Background And Objectives: Polycythemia vera (PV) and essential thrombocythemia (ET) are two rare acquired myeloproliferative disorders (MPD) with frequent thrombotic and hemorrhagic complications. The occurrence of thrombosis in unusual sites, e.g.