Sickle cell disease (SCD) is characterized by a complex vasculopathy, consisting of endothelial dysfunction and increased arterial stiffness, with a global effect on cardiovascular function. The hypercoagulable state may result from chronic hemolysis and circulating cell-derived microparticles (MPs) originating mainly from activated platelets and erythrocytes. We measured the levels of platelet and erythrocyte-derived MPs (PMPs and ErMPs) in 50 young SCD patients compared with 40 age- and sex-matched healthy controls and assessed their relation to clinicopathological characteristics and aortic elastic properties. Patients were studied stressing on the occurrence of sickling crisis, transfusion history, hydroxyurea therapy, hematological, and coagulation profile as well as flow cytometric expression of PMPs (CD41b(+)) and ErMPs (glycophorin A(+)). Echocardiography was performed to assess aortic stiffness and distensibility, left ventricular function and pulmonary artery pressure. Both PMPs and ErMPs were significantly elevated in SCD patients compared with control group (p < 0.001). SCD patients had significantly elevated d-dimer and von Willebrand factor antigen (vWF Ag) levels with lower antithrombin III compared with controls (p < 0.001). Aortic stiffness index and pulmonary artery pressure were significantly higher in SCD (p < 0.001), whereas aortic strain and aortic distensibility were significantly lower (p < 0.001) compared with controls. MPs levels were significantly increased in SCD patients with pulmonary hypertension, acute chest syndrome, and stroke as well as those who had history of thrombosis or splenectomy (p < 0.001). Also, patients in sickling crisis during the study had higher PMPs and ErMPs levels than those in steady state (p < 0.001). Patients on hydroxyurea therapy had lower MPs levels than untreated patients (p < 0.001). PMPs and ErMPs were positively correlated with disease duration, transfusion index, white blood cell count, HbS, markers of hemolysis, serum ferritin, D-dimer, and vWF Ag, whereas negatively correlated with hemoglobin and HbF levels (p < 0.05). Both PMPs and ErMPs levels were positively correlated with aortic stiffness, pulmonary artery pressure, and tricuspid regurgitant velocity (p < 0.05) while negatively correlated with aortic distensibility. We suggest that PMPs and ErMPs overproduction may be considered a potential biological marker for vascular dysfunction and disease severity in SCD and may be implicated in the pathogenesis of coagulation abnormalities encountered in those patients. Their levels are closely related to sickling crisis, pulmonary hypertension, markers of hemolysis, fibrinolysis, and iron overload. Therefore, quantification of MPs in SCD may provide utility for identifying patients who are at increased risk of thrombotic events or cardiovascular abnormalities and would help to monitor response to hydroxyurea therapy.

We aimed to study the endothelial dysfunction among children and adolescents with transfusion-dependent β-thalassemia using von Willebrand factor antigen (VWF:Ag) and flow cytometric analysis of circulating CD144(+) endothelial microparticles (EMPs) and endothelial progenitor cells (CD34(+)VEGFR2(+)) and assess their relation to iron overload, erythropoietin and chelation therapy as well as echocardiographic parameters and carotid intima-media thickness. The VWF:Ag, EMPs, and CD34(+)VEGFR2(+) cells were significantly higher among patients with β-thalassemia than controls (P < .001). The type of chelation and patients' compliance did not influence the results. No significant correlations were found between the studied vascular markers. Patients with evident heart disease had higher VWF: Ag, EMPs, and CD34(+)VEGFR2(+) cells than those without. Carotid intima-media thickness was increased among patients but not correlated with vascular markers. We suggest that procoagulant EMPs and VWF: Ag are involved in cardiovascular complications in patients with young β-thalassemia. CD34(+)VEGFR2(+) cells were further increased in response to tissue injury contributing to reendothelialization and neovascularization.

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Background A large spectrum of cardiac involvement in rheumatoid arthritis (RA) was already described. The most common cardiac involvement in RA is pericarditis [1]. RA-associated valvular heart disease is common [2]). Also RA patients had higher incidence of heart failure compared to general population due to left ventricular diastolic dysfunction [3], which remains clinically asymptomatic for a long time [4]. Objectives This study was designed to assess cardiac abnormalities in a population of Egyptian patients with rheumatoid arthritis, their association with cardiovascular risk factors and disease characteristics Methods Our study included 50 RA patients and 10 healthy individuals as controls. For all included subjects, detailed medical history was taken, general and local examination were performed as well as laboratory investigations including (complete blood count, ESR, BUN, serum creatinine, AST, ALT, HDL, LDL, cholesterol, triglycerides, hs-CRP, vWF, RF), ECG and echocardiography. RA disease activity was assessed using DAS28 (ESR). Results On Echocardiography, 2 (4%) patients had minimal pericardial effusion, 18 (36%) patient had diastolic dysfunction and 40 (80%) patients had valvular disease: 18 (36%) patients had MR, 6 (12%) patients had AR, 12 (24%) patients had TR and 4 (8%) patients had PR. Patients with valvular lesions were older than other patients (P<0.001). RA patients had significantly higher vWF, hs-CRP, ESR (p<0.001) and diastolic dysfunction prevalence (p=0.023) when compared to controls. Diastolic dysfunction was more in older patients and patients with longer disease duration, higher disease activity, higher hs-CRP (p<0.001), DM, hypertension, longer NSAIDS use and in presence MR, AR and pericardial effusion. vWF was not different (p=0.11) between those with diastolic dysfunction and those without diastolic dysfunction. hs-CRP ≥6.25 ug/ml had high sensitivity (82.4%) and low specificity (51.6%) in diagnosis of diastolic dysfunction in RA patients. Conclusions Patients with RA have significantly higher inflammatory markers (ESR, h-CRP and vWF) as compared to controls. Valvular lesions and diastolic dysfunction are common in RA patients. The prevalence of diastolic dysfunction in RA patients increases with older age, presence of DM, hypertension, longer disease duration, more disease activity, longer use of NSAIDS and high titre of hs-CRP. References Voskuyl AE (2006): The heart and cardiovascular manifestations in rheumatoid arthritis. Rheumatology Oxford; 45 Suppl 4:iv 4-7. Roldan CA, DeLong C, Qualls CR and Crawford MH (2007): Characterization of valvular heart disease in rheumatoid arthritis by transesophageal echocardiography and clinical correlates. Am J Cardiol; 100: (3), 496-502. Udayakumar N, Venkatesan S and Rajendiran C (2007): Diastolic function abnormalities in rheumatoid arthritis: relation with duration of disease. Singapore Med J; 48 (6):537. Philbin EF, Rocco TA, Lindenmuth NW, Ulrich K and Jenkins PL (2000): Systolic versus diastolic heart failure in community practi...