Ersi Voskaridou

Laiko Hospital, Athínai, Attica, Greece

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Publications (75)267.95 Total impact

  • Ersi Voskaridou · Maria Dimopoulou · Marouso Drosou
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    ABSTRACT: Please indicate your presentation preference: Oral Presentation Has the submitted material been published in a journal (printed or online)?: No Has the submitted material been presented or submitted to another event?: No Background: A spectrum of large clinical programs has established the efficacy and safety of the oral iron chelator deferasirox (DFX) in transfusion-dependent patients with iron overload. We report the results from a multicenter, prospective, open-label, observational study reflecting the standard clinical practice in thalassemia centers in Greece. Aims: To assess the safety profile of DFX in transfusion-dependent patients with iron overload. Methods: Patients with hemoglobinopathies and transfusional iron overload, baseline serum ferritin levels <4000 ng/mL, and for whom the physician decided to initiate chelation with DFX under the local approved label and standard medical practice, were included in the study. Patients with myelodysplastic syndromes, severe cardiac siderosis (T2* <10 ms), left ventricular ejection fraction <56%, serum creatinine (SrCr) >upper limit of normal (ULN), alanine aminotransferase (ALT) ≥500 U/L, creatinine clearance (CrCl) <60 mL/min or significant proteinuria were not eligible. Results: Of the 230 patients enrolled, 226 were evaluated (4 patients were excluded due to screen failures); a subset of the enrolled patients were pediatric (age <16 years, n=24). Mean age was 35.1±13.8 (pediatric, 8.7±4.2; adults, 38.3±10.9) years. The most common underlying conditions for transfusional iron overload were β-thalassemia major (66.8%, n=151), thalassemia intermedia (17.7%, n=40), and sickle cell anemia (9.3%, n=21). Prior chelation treatment was reported for 146 (64.6%) patients, most common being a combination of deferoxamine and deferiprone (19.0%; n=43). None of the patients had prior DFX exposure. Of the 226 evaluable patients, 179 completed the 12-month observation period, whereas 47 withdrew from the study prematurely. The 3 main reasons for discontinuation were adverse events (AEs; 7.1, n=16), lost to follow-up (4.4%, n=10), and consent withdrawal (2.2%, n=5). A total of 356 non-serious AEs were reported in 103 (45.6%) patients, of which 167 AEs observed in 65 patients (28.8%) were reported to be related to DFX. The most common non-serious AEs related to DFX were increase in SrCr above ULN (8.4%), upper abdominal pain (4.9%), and diarrhea (4.0%). Twenty-one (9.3%) patients experienced 56 serious AEs (SAEs), of which 13 SAEs in 3 patients (1.3%) were reported to be related to DFX, including gastrointestinal disorders (9
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  • No preview · Article · Jun 2015 · Human gene therapy. Clinical development
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    ABSTRACT: Objective The aim of this study was to evaluate bone involvement in patients with Gaucher disease (GD) and to propose a novel semi-quantitative magnetic resonance imaging (MRI) staging.MethodsMRI of the lumbar spine, femur and tibia was performed in 24 GD patients and 24 healthy controls. We also measured circulating levels of C-C motif ligand-3 (CCL-3) chemokine, C-telopeptide of collagen type-1 (CTX) and tartrate resistant acid phosphatase isoform type-b (TRACP-5b).ResultsWe used the following staging based on MRI data: stage I: region of interest (ROI) 1/2 of normal values and bone infiltration up to 30%; stage II: ROI 1/3 of normal values and bone infiltration from 30 to 60%; stage III: ROI 1/4 of normal values and bone infiltration from 60% to 80% and stage IV: detection of epiphyseal infiltration, osteonecrosis and deformity regardless of the ROI's values. All but 2 patients had abnormal MRI findings: 9 (37.5%), 6 (25%), 3 (12.5%) and 4 (16.7%) had stages I-IV, respectively. GD patients had elevated chitotriosidase, serum TRACP-5b and CCL-3 levels (p<0.001).Conclusions We propose an easily reproducible semi-quantitative scoring system and confirm that GD patients have abnormal MRI bone findings and enhanced osteoclast activity possibly due to elevated CCL-3.This article is protected by copyright. All rights reserved.
    No preview · Article · Jan 2015 · European Journal Of Haematology
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    ABSTRACT: Objective Progress in the management of patients with thalassaemia intermedia (TI) enabled increasing rates of pregnancies among TI women worldwide. Nevertheless, information regarding TI pregnancy management and outcome is quite limited in the literature. The aim of this study was to report our experience regarding the maternal and fetal outcome of TI patients, as well as to depict the complexity of the disease and the need for multidisciplinary and personalized management as shown by the description of two interesting pregnancy cases.Methods We analysed our data recorded from 60 pregnancies in 34 women over a 20 year period.ResultsForty nine patients achieved full-term pregnancies (mean maternal age±SD: 27.4±6.5 years) within 37±3 gestation weeks. Their mean haemoglobin value was 8.33±1.22 g/dl; 26.5% of patients were not transfused at all or they had been transfused only once during gestation. There were eleven abortions (18.3%). The spontaneous abortions (5/11) were related to high HbF levels. Six patients had more than two normal deliveries. Nineteen newborns (38.8%), which weighed 2-3 kg, required hospitalization to an intensive neonatal care unit for 1-3 weeks. One patient presented with life-threatening complications (haemolytic anaemia, thrombocytopenia and enlargement of spleen) and another with spastic paraparesis due to extramedullary paravertebral masses.Conclusions Although several complications can occur during a pregnancy in TI women, the careful and frequent monitoring by both haematologists and obstetricians can lead to successful deliveries.This article is protected by copyright. All rights reserved.
    No preview · Article · May 2014 · European Journal Of Haematology
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    ABSTRACT: Iron overload is a common complication of patients with β-thalassemia major (TM). Despite the availability of three iron chelators, deferoxamine (DFO), deferiprone (DFP), and deferasirox (DFX), some patients fail to respond adequately to monotherapy with any of them. We report a case of TM who had refractory severe iron overload and was successfully and safely chelated with the combination of DFX with DFO. A 40-year-old male with β-TM, who had been regularly transfused from the age of 2, had been administered in the past iron chelation with DFO, DFP, and DFX monotherapy, without major improvement on his iron overload status. Liver and cardiac magnetic resonance imaging (MRI) revealed severe iron overload, while serum ferritin was persistently greater than 2500 μg/L. After the patient gave informed consent, he was administered combination therapy of DFX at 30 mg/kg/day for 7 days per week and DFO at 2500 mg/day for 4 days every week and routinely followed up for compliance. Eighteen months later, serum ferritin was reduced to 680 μg/L, while both liver and cardiac MRI T2* values improved, reflecting lower iron overload. The combination regimen was well tolerated and no adverse events were documented. This is the first official report of simultaneous daily administration of the two iron chelators DFX and DFO that demonstrates the beneficial effect of the combination on heart and liver hemosiderosis. If our observation is confirmed in more patients, this combination could constitute a useful option in tailoring individual chelation therapy for β-TM patients with iron overload.
    No preview · Article · Jul 2013 · Transfusion
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    ABSTRACT: Background. HCV infection and transfusional iron overload in Thalassemic patients may result in liver disease. HCV treatment in Thalassemia has raised safety concerns. Aim. Estimate effectiveness and tolerability of interferon-based therapy in HCV-infected Thalassemic patients. Material and methods. Over a 12-year period, consecutive patients with β Thalassemia major (TM) and chronic hepatitis C received treatment. Liver biopsy, HCV-RNA and genotyping were performed beforehand. Sustained virological response (SVR) was defined as negative HCV-RNA 6 months post-treatment. Forty eight patients (26 M-22 F, mean age 39.8) were enrolled. Twenty nine patients were treated with conventional interferon alpha (IFNa) for 48 weeks (group A). Nineteen patients (10 naïve-9 previously IFNa experienced) received pegylated interferon (PEGIFN) (group B). Results. HCV-1 was found in 44%, HCV-2 in 14%, HCV-3 in 23% and HCV-4 in 19%. Group A: ten patients (38.5%) achieved SVR, 2 (7.5%) relapsed and 17 (54%) were non responders. Group B: five (28%) achieved SVR, 8 (44%) relapsed and 6 (28%) never responded. High HCV-RNA levels, genotype 1 and advanced liver fibrosis were independently associated with no response. Four patients (3 treated with IFNα, 1 with PEG-IFN) had to discontinue treatment due to complications. Conclusions. The response rate of IFN monotherapy in multi-transfused, HCV-infected Thalassemic patients is not inferior to that in non-multitransfused patients. IFNa administration is well-tolerated and should be recommended as initial treatment schedule in this setting.
    No preview · Article · Jul 2013 · Annals of hepatology: official journal of the Mexican Association of Hepatology
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    Ersi Voskaridou · Evangelos Terpos

    Preview · Article · Mar 2013
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    ABSTRACT: Iron overload and hepatitis-C virus (HCV) infection, have been implicated in the evolution of liver disease, in patients with transfusion-dependent beta-thalassaemia major (BTM). However, the impact of these factors in late stages of liver disease in adults with BTM, has not been extensively studied. To investigate serum indices of iron overload, HCV infection and liver disease, in a cohort of 211 adult Greek patients with BTM, in relation with the findings from liver biopsies. In this cross-sectional study, 211 patients with BTM were enrolled and studied, in relation with HCV infection, ferritin, transaminases, chelation treatment and antiviral treatment. Based on 109 patients biopsied, we correlated liver fibrosis, haemosiderosis and inflammation, with serum indices and HCV status Among all patients, 74.4% were anti-HCV positive (HCV+). Ferritin was positively correlated with transaminases and negatively correlated with age, while it was not significantly different among HCV+ and HCV- patients. Among the HCV+ patients, 55.4% reported antiviral treatment, while genotype 1 predominated. In a subfraction of 109 patients, in which liver biopsy was performed, 89% were HCV+ and 11% HCV-. Fibrosis was significantly correlated with age (P = 0.046), AST (P = 0.004), ALT (P = 0.044) and inflammation (P < 0.001). Advanced fibrosis was present with even minimal haemosiderosis, independently of ferritin values or HCV history. These data suggest that in the late stages of liver disease in BTM patients, iron overload may be the critical determinant, since fibrosis is related to the minimal haemosiderosis, independently of HCV history.
    Full-text · Article · Mar 2013 · Liver international: official journal of the International Association for the Study of the Liver
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    Preview · Article · Dec 2012 · Turkish Journal of Haematology
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    ABSTRACT: Osteoporosis is a severe complication of thalassemia. Sclerostin is a Wnt signaling inhibitor, which is produced by osteocytes and inhibits osteoblast function. Sclerostin is implicated in the pathogenesis of osteoporosis of different etiology. The aim of the study was to evaluate circulating sclerostin in 66 patients (median age 42 years) with thalassemia and osteoporosis who participated in a phase 2, randomized study (zoledronic acid vs. placebo) and the results were compared with those of 30 healthy controls (median age 44 years) without osteopenia/osteoporosis and 62 women with postmenopausal osteoporosis (median age 63 years). At baseline, thalassemic patients with osteoporosis had elevated circulating levels of sclerostin (median: 605 pg/ml, range: 22-1 227 pg/ml) compared to healthy controls without osteopenia/osteoporosis (250 pg/ml, 0-720 pg/ml, p<0.001) and reduced levels of sclerostin compared with postmenopausal women with osteoporosis (840 pg/ml, 181-1 704 pg/ml, p<0.001). Thalassemia patients had also increased serum dickkopf-1 (Dkk-1) and high bone turnover. Circulating sclerostin levels correlated with bone mineral density in lumbar spine (r=0.619, p<0.001), distal radius (r=0.401, p=0.001) and femoral neck (r=0.301, p=0.021). Zoledronic acid did not alter sclerostin levels after 12 months of therapy, although it reduced circulating Dkk-1. We conclude that circulating sclerostin is elevated in thalassemia patients with osteoporosis and correlated with their BMD, but it was not reduced post zoledronic acid administration. These findings suggest that high sclerostin may serve as a marker of increased osteocyte activity in thalassemia patients. Drugs targeting sclerostin may also be used in this difficult to treat disorder associated with bone loss.
    No preview · Article · May 2012 · Hormone and Metabolic Research
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    Ersi Voskaridou · Dimitrios Christoulas · Evangelos Terpos
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    ABSTRACT: Sickle cell disease (SCD) is an inherited chronic haemolytic anaemia whose clinical manifestations arise from the tendency of the haemoglobin to polymerize and deform red blood cells into the characteristic sickle shape due to a single nucleotide change in the β-globin. Vascular occlusion of small and large vessels can lead to chronic damage of multiple organs including brain, lung, bone, kidney, liver, spleen, and retina. However, the extent to which SCD impacts myocardial function is not very clear. Cardiovascular manifestations include both right and left ventricular systolic and diastolic dysfunction, elevated cardiac output, cardiomegaly and myocardial ischaemia. Progressive heart damage from iron overload occurs in patients requiring routine transfusion therapy. Pulmonary hypertension resulting from intravascular haemolysis has also been recognized as a major complication that independently correlates with survival. This review summarizes all available data for the heart complications in SCD to update the physicians for their appearance, diagnostic procedures and possible management.
    Full-text · Article · Apr 2012 · British Journal of Haematology
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    ABSTRACT: Haemoglobinopathies are the most common hereditary disorders in Greece. Although there is a successful national prevention program, established 35 years ago, there is lack of an official registry and collection of epidemiological data for haemoglobinopathies. This paper reports the results of the first National Registry for Haemoglobinopathies in Greece (NRHG), recently organized by the Greek Society of Haematology. NRHG records all patients affected by thalassaemia major (TM), thalassaemia intermedia (TI), "H" Haemoglobinopathy (HH) and sickle cell disease (SCD). Moreover, data about the annual rate of new affected births along with deaths, between 2000 and 2010, are reported. A total of 4,506 patients are registered all over the country while the number of affected newborns was significantly decreased during the last 3 years. Main causes for still having affected births are: (1) lack of medical care due to financial reasons or low educational level; (2) unawareness of time limitations for prenatal diagnosis (PD); due either to obstetricians' malpractice or to delayed demand of medical care of couples at risk; and (3) religious, social or bioethical reasons. Cardiac and liver disorders consist main causes for deaths while life expectancy of patients lengthened after 2005 (p < 0.01). The NRHG of patients affected by haemoglobinopathies in Greece provides useful data about the haemoglobinopathies in the Greek population and confirms the efficacy of the National Thalassaemia Prevention Program on impressively decreasing the incidence of TM and sickle cell syndromes.
    Full-text · Article · Apr 2012 · Annals of Hematology
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    D. Loukopoulos · E. Voskaridou · M. Tsalkani

    Preview · Article · Jan 2012
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    Ersi Voskaridou · Dimitrios Christoulas · Evangelos Terpos

    Full-text · Article · May 2011 · British Journal of Haematology
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    ABSTRACT: Iron overload is present in several cases of double heterozygous sickle-cell/beta-thalassemia (HbS/β-thal). Deferasirox is an orally administered iron chelator which is effective on iron overloaded patients with transfusion-dependent anemia. The aim of this study was to investigate the efficacy and safety of deferasirox on HbS/β-thal patients with iron overload. We evaluated 31 adult patients with HbS/β-thal (14M/17F; median age 41 years) who had serum ferritin levels >1,000 ng/mL and who were sporadically transfused. Total iron burden was monitored by measuring serum ferritin levels before and monthly after starting deferasirox, while liver iron concentration and cardiac iron burden were measured by magnetic resonance imaging (MRI) T2 and T2* parameters at baseline and 12 months after deferasirox treatment. Deferasirox managed to reduce the mean serum ferritin levels after 12 months of treatment from 1,989 ± 923 to 1,008 ± 776 ng/mL (P < 0.001). This reduction was accompanied by a significant improvement on MRI T2* of the liver (from 3.9 ± 3.2 to 5.8 ± 3.1 ms; P < 0.01) and by a comparable improvement of biochemical parameters of liver function. Mild nausea and diarrhea of grade 1/2 were reported in 25% of patients within the first month of treatment, but did not re-occur. These data indicate that deferasirox provided effective control of iron levels (mainly of the liver) in minimally transfused patients with HbS/β-thal, without significant adverse events, at similar doses to those studied widely for the treatment of patients with thalassemia syndromes.
    Full-text · Article · Jan 2011 · Annals of Hematology
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    Evangelos Terpos · Ersi Voskaridou
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    ABSTRACT: Osteoporosis represents a prominent cause of morbidity in patients with thalassemia. The delay in sexual maturation, the presence of diabetes and hypothyroidism, the parathyroid gland dysfunction, the progressive marrow expansion, the iron toxicity on osteoblasts, the iron chelators, and the deficiency of growth hormone or insulin growth factors have been identified as major causes of osteoporosis in thalassemia. Adequate hormonal replacement, effective iron chelation, improvement of hemoglobin levels, calcium and vitamin D administration, physical activity, and smoking cessation are the main to-date measures for the management of the disease. During the last decade, novel pathogenetic data suggest that the reduced osteoblastic activity, which is believed to be the basic mechanism of bone loss in thalassemia, is accompanied by a comparable or even greater increase in bone resorption. Therefore, the role of bisphosphonates, potent inhibitors of osteoclast activation, arises as a major factor in the management of osteoporosis in thalassemia patients.
    Full-text · Article · Aug 2010 · Annals of the New York Academy of Sciences
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    ABSTRACT: The aim of this prospective study was to evaluate the long-term efficacy and safety of hydroxyurea (HU) in patients with sickle cell disease (SCD). Thirty-four patients with sickle cell anemia (hemoglobin S [HbS]/HbS), 131 with HbS/beta(0)-thal, and 165 with HbS/beta(+)-thal participated in this trial. HU was administered to 131 patients, whereas 199 patients were conventionally treated. The median follow-up period was 8 years for HU patients and 5 years for non-HU patients. HU produced a dramatic reduction in the frequency of severe painful crises, transfusion requirements, hospital admissions, and incidence of acute chest syndrome. The probability of 10-year survival was 86% and 65% for HU and non-HU patients, respectively (P = .001), although HU patients had more severe forms of SCD. The 10-year probability of survival for HbS/HbS, HbS/beta (0)-thal, and HbS/IVSI-110 patients was 100%, 87%, and 82%, respectively, for HU patients and 10%, 54%, and 66%, for non-HU patients. The multivariate analysis showed that fetal hemoglobin values at baseline and percentage change of lactate dehydrogenase between baseline and 6 months were independently predicted for survival in the HU group. These results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients.
    Preview · Article · Nov 2009 · Blood
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    Full-text · Article · Oct 2009 · British Journal of Haematology
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    ABSTRACT: Hemoglobin (Hb) disorders are common, potentially lethal monogenic diseases, posing a global health challenge. With worldwide migration and intermixing of carriers, demanding flexible health planning and patient care, hemoglobinopathies may serve as a paradigm for the use of electronic infrastructure tools in the collection of data, the dissemination of knowledge, the harmonization of treatment, and the coordination of research and preventive programs. ITHANET, a network covering thalassemias and other hemoglobinopathies, comprises 26 organizations from 16 countries, including non-European countries of origin for these diseases (Egypt, Israel, Lebanon, Tunisia and Turkey). Using electronic infrastructure tools, ITHANET aims to strengthen cross-border communication and data transfer, cooperative research and treatment of thalassemia, and to improve support and information of those affected by hemoglobinopathies. Moreover, the consortium has established the ITHANET Portal, a novel web-based instrument for the dissemination of information on hemoglobinopathies to researchers, clinicians and patients. The ITHANET Portal is a growing public resource, providing forums for discussion and research coordination, and giving access to courses and databases organized by ITHANET partners. Already a popular repository for diagnostic protocols and news related to hemoglobinopathies, the ITHANET Portal also provides a searchable, extendable database of thalassemia mutations and associated background information. The experience of ITHANET is exemplary for a consortium bringing together disparate organizations from heterogeneous partner countries to face a common health challenge. The ITHANET Portal as a web-based tool born out of this experience amends some of the problems encountered and facilitates education and international exchange of data and expertise for hemoglobinopathies.
    Full-text · Article · Sep 2009 · Hemoglobin
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    ABSTRACT: Dickkopf-1 is an inhibitor of Wnt signaling, which is crucial for osteoblast differentiation. We evaluated serum levels of Dickkopf-1 in 66 patients with thalassemia-induced osteoporosis who received therapy with zoledronic acid in a placebo-controlled, randomized trial. At baseline, thalassemia patients had increased serum levels of Dickkopf-1 that correlated with reduced bone mineral density of the lumbar spine and the distal radius. High Dickkopf-1 also correlated with increased bone resorption and reduced bone formation markers. Zoledronic acid produced a reduction in serum Dickkopf-1, which was associated with bone mineral density increase after 12 months of therapy. On the contrary, placebo group showed a borderline increase of Dickkopf-1, which was higher in patients who showed deterioration in pain scores. These results suggest that Dickkopf-1 is implicated in the pathogenesis of osteoporosis in thalassemia and reveal Dickkopf-1 as a possible target for the development of novel agents for the management of thalassemia-induced osteoporosis.
    Full-text · Article · Jun 2009 · Haematologica