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Ashok Raj - One of the best experts on this subject based on the ideXlab platform.
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long term Erythrocytapheresis is associated with reduced liver iron concentration in sickle cell disease
Journal of Pediatric Hematology Oncology, 2016Co-Authors: Scott N. Myers, Salvatore Bertolone, Ryan Eid, John Myers, Arun Ranjan Panigrahi, Jennifer Mullinax, Ashok RajAbstract:BACKGROUND Erythrocytapheresis procedures are increasingly used in sickle cell disease. Serum ferritin and noninvasive magnetic resonance imaging measurements of liver iron concentration (LIC) are frequently used to monitor iron overload secondary to hypertransfusion. There is a paucity of data describing the impact of long-term Erythrocytapheresis (LTE) on LIC. MATERIALS AND METHODS We measured magnetic resonance imaging liver and cardiac iron on LTE subjects and stratified them into 2 groups: higher LIC (>3 mg/g) and lower LIC (<3 mg/g). χ(2) and t test were used to test for differences between the 2 groups. Logistic regression and generalized linear mixed-effects models were used to test what impacted LIC. RESULTS None of 29 sickle cell disease subjects maintained on LTE had high cardiac iron concentration. LIC was associated with serum ferritin (r=0.697, P<0.001) but was not associated with the total number of LTE procedures (r=-0.088, P=0.656) or total number of simple transfusions (r=0.316, P=0.108). The total number of LTE procedures was not associated with serum ferritin (r=0.040, P=0.838), the total number of simple transfusions (r=-0.258, P=0.184), or LIC group (r=-0.111, P=0.566). CONCLUSION There was no significant correlation between duration of LTE maintenance and LIC.
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Long-term Erythrocytapheresis Is Associated With Reduced Liver Iron Concentration in Sickle Cell Disease.
Journal of Pediatric Hematology Oncology, 2016Co-Authors: Scott N. Myers, Salvatore Bertolone, Ryan Eid, John Myers, Arun Ranjan Panigrahi, Jennifer Mullinax, Ashok RajAbstract:BACKGROUND Erythrocytapheresis procedures are increasingly used in sickle cell disease. Serum ferritin and noninvasive magnetic resonance imaging measurements of liver iron concentration (LIC) are frequently used to monitor iron overload secondary to hypertransfusion. There is a paucity of data describing the impact of long-term Erythrocytapheresis (LTE) on LIC. MATERIALS AND METHODS We measured magnetic resonance imaging liver and cardiac iron on LTE subjects and stratified them into 2 groups: higher LIC (>3 mg/g) and lower LIC (
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Utility of impedance cardiography for the detection of hemodynamic changes in stable patients with sickle cell disease
Journal of Pediatric Hematology Oncology, 2012Co-Authors: Bibhuti B. Das, Maiying Kong, Ashok Raj, Michael R. Recto, Salvatore BertoloneAbstract:OBJECTIVES The study sought to assess the potential utility of impedance cardiography (ICG) to detect hemodynamic changes after Erythrocytapheresis in stable children with sickle cell disease (SCD). METHODS We prospectively monitored cardiac index, systemic vascular resistance index, heart rate, and blood pressure using ICG before and after Erythrocytapheresis in 26 stable children with SCD. Echocardiography was carried out in all patients to evaluate left ventricular systolic function. Hemoglobin (Hb), sickle cell hemoglobin (HbS), and ferritin levels were also measured. RESULTS Of a total of 78 Erythrocytapheresis procedures in 26 children with SCD, 22 (28.2%) had hypotensive episodes defined as a decrease in systolic, diastolic, or mean blood pressure by 10 mmHg. Risk factors for developing hypotension during Erythrocytapheresis were identified with logistic regression analysis: lower-body surface area and decrease in cardiac index. In contrast, age, prepheresis Hb and HbS, serum ferritin levels, and left ventricular function at baseline were not associated with hypotension. CONCLUSIONS This study demonstrates the feasibility of the ICG technique to detect the hemodynamic changes in children with SCD after an Erythrocytapheresis procedure.
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Antibody development in pediatric sickle cell patients undergoing Erythrocytapheresis.
Pediatric Blood & Cancer, 2010Co-Authors: Gwendolyn J Godfrey, Salvatore Bertolone, Maiying Kong, William Lockwood, Ashok RajAbstract:Background Erythrocytapheresis, or red blood cell exchange transfusion (RBCX), with donor red blood cell (RBC) units is now increasingly used in the treatment of acute and chronic complications of sickle cell disease (SCD). As in all transfusions, RCBX carries a risk of immunization against foreign antigen on transfused cells. However, by selecting donor units with RBC phenotypes similar to the patient, the risk of allo- and autoimmunization can be reduced. Procedure The formation of RBC alloantibodies and/or autoantibodies in 32 multitransfused pediatric SCD patients undergoing monthly RBCX over a 11-year period (12/1998 to 12/2009) was evaluated utilizing a retrospective patient chart review at Kosair Children's Hospital, Louisville, Kentucky. Results After starting C, E, K antigen-matched RBCX, the rate of clinically significant allo-immunization decreased from 0.189/100 to 0.053/100 U, with a relative risk of 27.9%. Likewise, the rate of autoimmunization decreased from 0.063/100 to 0.035/100 U, with a relative risk of 55.9%. Conclusion After controlling for clinically insignificant antibodies, our auto- and alloimmunization rate was much less than previously reported values. In addition, the incidence of clinically significant allo- and autoimmunization decreased in our patient population after starting minor antigen-matched RBCX. These results suggest that by matching selected RBC phenotypes, there may be an association in the risk of allo- and autoimmunization of multi-transfused SCD patients. Pediatr Blood Cancer. 2010;55:1134–1137. © 2010 Wiley-Liss, Inc.
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Hemodynamic Monitoring during Erythrocytapheresis in Sickle Cell Disease Patients Using Impedance Cardiography
Blood, 2008Co-Authors: Bibhuti B. Das, Samyuktha Kemparajurs, Salvatore Bertolone, Maiying Kong, Ashok RajAbstract:BACKGROUND: Erythrocytapheresis procedures are increasingly used in the treatment of severe acute complications of sickle cell disease (SCD). Some patients who undergo Erythrocytapheresis experience dizziness during or after the procedure. There is no data in literature for hemodynamic monitoring of cardiac index (CI) and systemic vascular resistance index (SVRI) during Erythrocytapheresis. We have previously shown that impedance cardiography (ICG) and invasive measures of CI by thermodilution techniques are significantly correlated in children. OBJECTIVES: We wanted to evaluate changes in CI and SVRI using ICG and to correlate these with systolic blood pressure (SBP) measurements in SCD patients while undergoing Erythrocytapheresis. METHODS: Thirty one SCD patients who had 70 consecutive Erythrocytapheresis procedures were included in this IRB approved study. ICG measurements (CI, SVRI, and blood pressure) were taken pre-Erythrocytapheresis, 30 minutes after initiation of the procedure, one hour into Erythrocytapheresis, and at the end of the procedure. ICG measurements were repeated in symptomatic patients before and after intervention with fluid administration. RESULTS: We evaluated hemodynamic data on 31 SCD patients, average age 12.8±5.2 years and 56% are female. SBP decreased below the base line during 33 procedures and increased above the baseline during 37 procedures. Among patients who had a decrease in SBP, 28/33 (84%) had decreased CI. Among them 22/28 (78%) had increased SVRI. Among patients who had an increase in SBP, 5/37 (14%) had increased CI. Among them 4/5 (80%) also had increased SVRI. Using paired t test, the changes in CI (P CONCLUSIONS: In summary, the hemodynamic changes during Erythrocytapheresis are probably multifactorial in origin and may include a combination of SVRI and CI changes. Confirmation of these findings may allow early intervention in some patients and minimize the risk of developing overt clinical symptoms during Erythrocytapheresis.
Gisèle Soubrane - One of the best experts on this subject based on the ideXlab platform.
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Hemodilution therapy using automated Erythrocytapheresis in central retinal vein occlusion: results of a multicenter randomized controlled study
Graefe's Archive for Clinical and Experimental Ophthalmology, 2011Co-Authors: Agnès Glacet-bernard, Marielle Atassi, Christine Fardeau, Jean-paul Romanet, Matthieu Tonini, John Conrath, Philippe Denis, Martine Mauget-faÿsse, Gabriel Coscas, Gisèle SoubraneAbstract:Background Central retinal vein occlusion (CRVO) leads to poor visual outcome in most eyes. Abnormal hemorheology was suspected to play a major role in its pathogenesis. CRVO treatment is still a matter of debate but several studies have pointed out the efficacy of isovolumic hemodilution. The aim of this study was to assess the feasibility and efficacy of hemodilution using automated Erythrocytapheresis in recent-onset CRVO. Methods In this prospective randomized controlled multicenter study, 61 consecutive CRVO patients were enrolled when they met the following criteria: CRVO lasting for 3 weeks or less, visual acuity ranging from 20/200 to 20/32, age between 18 and 85 years, no diabetes, no uncontrolled systemic hypertension, no antiplatelet or anticoagulant therapy, hematocrit higher than 38%, and signed informed consent. Patients were randomly assigned to the hemodilution group ( n = 31) or to the control group ( n = 30). Hemodilution therapy consisted of one session of Erythrocytapheresis on outpatient basis, followed by additional session(s) for 6 weeks if needed. Target hematocrit was 35%. Follow-up was 12 months. Results No statistical differences in age, associated risk factors, or CRVO characteristics were observed at baseline between both groups. Mean visual acuity was equivalent to 20/80 in the hemodilution group and to 20/63 in the control group (non-significant difference). In the treated group, mean number of hemodilution sessions was 3.3 (range, 1 to 6), and no major side-effects occurred. At the 12-month follow-up visit, 64.5% of the hemodilution group had visual acuity of 20/40 or better compared to 40% of the control group ( p = .048). Visual change was a gain of 1.7 ETDRS line in the hemodilution group versus a loss of 2.3 lines in the control group ( p = .007). There was less conversion into an ischemic form in the hemodilution group (11%) than in the control group (50%, p = .004). Mean final retinal thickness was 289 μm in the hemodilution group versus 401 μm in the control group ( p = .068). Conclusions This multicenter controlled randomized study demonstrated that automated Erythrocytapheresis is a safe and effective tool for performing hemodilution and confirmed that hemodilution therapy can improve the final prognosis of CRVO when applied in the early phase of the disease.
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Hemodilution therapy using automated Erythrocytapheresis in central retinal vein occlusion: results of a multicenter randomized controlled study
Graefe's Archive for Clinical and Experimental Ophthalmology, 2011Co-Authors: Agnès Glacet-bernard, Marielle Atassi, Christine Fardeau, Jean-paul Romanet, Matthieu Tonini, John Conrath, Philippe Denis, Martine Mauget-faÿsse, Gabriel Coscas, Gisèle SoubraneAbstract:Background Central retinal vein occlusion (CRVO) leads to poor visual outcome in most eyes. Abnormal hemorheology was suspected to play a major role in its pathogenesis. CRVO treatment is still a matter of debate but several studies have pointed out the efficacy of isovolumic hemodilution. The aim of this study was to assess the feasibility and efficacy of hemodilution using automated Erythrocytapheresis in recent-onset CRVO. Methods In this prospective randomized controlled multicenter study, 61 consecutive CRVO patients were enrolled when they met the following criteria: CRVO lasting for 3 weeks or less, visual acuity ranging from 20/200 to 20/32, age between 18 and 85 years, no diabetes, no uncontrolled systemic hypertension, no antiplatelet or anticoagulant therapy, hematocrit higher than 38%, and signed informed consent. Patients were randomly assigned to the hemodilution group ( n = 31) or to the control group ( n = 30). Hemodilution therapy consisted of one session of Erythrocytapheresis on outpatient basis, followed by additional session(s) for 6 weeks if needed. Target hematocrit was 35%. Follow-up was 12 months. Results No statistical differences in age, associated risk factors, or CRVO characteristics were observed at baseline between both groups. Mean visual acuity was equivalent to 20/80 in the hemodilution group and to 20/63 in the control group (non-significant difference). In the treated group, mean number of hemodilution sessions was 3.3 (range, 1 to 6), and no major side-effects occurred. At the 12-month follow-up visit, 64.5% of the hemodilution group had visual acuity of 20/40 or better compared to 40% of the control group ( p = .048). Visual change was a gain of 1.7 ETDRS line in the hemodilution group versus a loss of 2.3 lines in the control group ( p = .007). There was less conversion into an ischemic form in the hemodilution group (11%) than in the control group (50%, p = .004). Mean final retinal thickness was 289 μm in the hemodilution group versus 401 μm in the control group ( p = .068). Conclusions This multicenter controlled randomized study demonstrated that automated Erythrocytapheresis is a safe and effective tool for performing hemodilution and confirmed that hemodilution therapy can improve the final prognosis of CRVO when applied in the early phase of the disease.
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Hemodilution therapy using automated Erythrocytapheresis in central retinal vein occlusion: results of a multicenter randomized controlled study.
Graefe's Archive for Clinical and Experimental Ophthalmology, 2010Co-Authors: Agnès Glacet-bernard, Marielle Atassi, Christine Fardeau, Jean-paul Romanet, Matthieu Tonini, John Conrath, Philippe Denis, Martine Mauget-faÿsse, Gabriel Coscas, Gisèle SoubraneAbstract:Background Central retinal vein occlusion (CRVO) leads to poor visual outcome in most eyes. Abnormal hemorheology was suspected to play a major role in its pathogenesis. CRVO treatment is still a matter of debate but several studies have pointed out the efficacy of isovolumic hemodilution. The aim of this study was to assess the feasibility and efficacy of hemodilution using automated Erythrocytapheresis in recent-onset CRVO.
Ger H Koek - One of the best experts on this subject based on the ideXlab platform.
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How we manage patients with hereditary haemochromatosis
British Journal of Haematology, 2016Co-Authors: Eva Rombout-sestrienkova, Marian G. J. Van Kraaij, Ger H KoekAbstract:A number of disorders cause iron overload: some are of genetic origin, such as hereditary haemochromatosis, while others are acquired, for instance due to repeated transfusions. This article reviews the treatment options for hereditary haemochromatosis, with special attention to the use of Erythrocytapheresis. In general, therapy is based on the removal of excess body iron, for which ferritin levels are used to monitor the effectiveness of treatment. For many decades phlebotomy has been widely accepted as the standard treatment. Recent publications suggest that Erythrocytapheresis, as a more individualized treatment, can provide a good balance between effectiveness, tolerability and costs. Other treatments like oral chelators and proton pomp inhibitors, which are used in selected patients, create the possibility to further individualize treatment of hereditary haemochromatosis. In the future, hepcidin-targeted therapy could provide a more fundamental approach to treatment.
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course of iron parameters in hfe hemochromatosis patients during initial treatment with Erythrocytapheresis compared to phlebotomy
Journal of Clinical Apheresis, 2016Co-Authors: Eva Romboutsestrienkova, Ger H Koek, Rabin E. J. Neslo, Marian G. J. Van Kraaij, Paul P.c.a. Menheere, Ad Masclee, Dorine W. SwinkelsAbstract:Current treatment for newly diagnosed patients with hereditary hemochromatosis (HH) and iron overload consist of weekly phlebotomy or less frequent and more personalized Erythrocytapheresis. Previous observations during phlebotomy suggest an increase in intestinal iron uptake caused by lowering of hepcidin as a result of intensive bloodletting. It is not known whether such an effect is present or even more pronounced using Erythrocytapheresis since a larger amount of iron is extracted per procedure. In this study we aimed to assess the effect of Erythrocytapheresis on the course of iron parameters, with special focus on serum hepcidin. We performed a retrospective proof-of-principle observational study, comparing serum iron parameters in 12 males during the depletion phase using either phlebotomy (n = 6) or Erythrocytapheresis (n = 6). Decreases in serum ferritin over time were similar for both treatments but more pronounced using Erythrocytapheresis when expressed per treatment procedure. Hemoglobin did not change during Erythrocytapheresis, whereas during phlebotomy decreased with 10%. Increase of erythropoietin and soluble transferrin receptor and decrease in transferrin saturation were similar for both treatments. Reduction in serum hepcidin was higher (50% versus 25% of initial value) and occurred more early using phlebotomy (10 versus 20 weeks after start). In aggregate, compared to phlebotomy, the less frequent and more personalized Erythrocytapheresis leads to a more pronounced decrease in serum ferritin per treatment procedure, without a larger decrease in serum hepcidin. This may be clinically relevant and may prevent an increase in intestinal iron uptake and an ensuing vicious circle of more frequent treatment procedures. J. Clin. Apheresis 31:564-570, 2016. © 2015 Wiley Periodicals, Inc.
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Course of iron parameters in HFE‐hemochromatosis patients during initial treatment with Erythrocytapheresis compared to phlebotomy
Journal of Clinical Apheresis, 2016Co-Authors: Eva Rombout-sestrienkova, Ger H Koek, Rabin E. J. Neslo, Marian G. J. Van Kraaij, Paul P.c.a. Menheere, Ad Masclee, Dorine W. SwinkelsAbstract:Current treatment for newly diagnosed patients with hereditary hemochromatosis (HH) and iron overload consist of weekly phlebotomy or less frequent and more personalized Erythrocytapheresis. Previous observations during phlebotomy suggest an increase in intestinal iron uptake caused by lowering of hepcidin as a result of intensive bloodletting. It is not known whether such an effect is present or even more pronounced using Erythrocytapheresis since a larger amount of iron is extracted per procedure. In this study we aimed to assess the effect of Erythrocytapheresis on the course of iron parameters, with special focus on serum hepcidin. We performed a retrospective proof-of-principle observational study, comparing serum iron parameters in 12 males during the depletion phase using either phlebotomy (n = 6) or Erythrocytapheresis (n = 6). Decreases in serum ferritin over time were similar for both treatments but more pronounced using Erythrocytapheresis when expressed per treatment procedure. Hemoglobin did not change during Erythrocytapheresis, whereas during phlebotomy decreased with 10%. Increase of erythropoietin and soluble transferrin receptor and decrease in transferrin saturation were similar for both treatments. Reduction in serum hepcidin was higher (50% versus 25% of initial value) and occurred more early using phlebotomy (10 versus 20 weeks after start). In aggregate, compared to phlebotomy, the less frequent and more personalized Erythrocytapheresis leads to a more pronounced decrease in serum ferritin per treatment procedure, without a larger decrease in serum hepcidin. This may be clinically relevant and may prevent an increase in intestinal iron uptake and an ensuing vicious circle of more frequent treatment procedures. J. Clin. Apheresis 31:564-570, 2016. © 2015 Wiley Periodicals, Inc.
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Erythrocytapheresis versus phlebotomy in the initial treatment of hfe hemochromatosis patients results from a randomized trial
Transfusion, 2012Co-Authors: Eva Romboutsestrienkova, Fred H M Nieman, Brigitte A B Essers, Paulus A H Van Noord, Mirian C H Janssen, Cees Th B M Van Deursen, Ferdinand Rombout, Rogier Van Den Braak, Peter W De Leeuw, Ger H KoekAbstract:BACKGROUND: Standard treatment of newly diagnosed HFE hemochromatosis patients is phlebotomy. Erythrocytapheresis provides a new therapeutic modality that can remove up to three times more red blood cells per single procedure and could thus have a clinical and economic benefit. STUDY DESIGN AND METHODS: To compare the number of treatment procedures between Erythrocytapheresis and phlebotomy needed to reach the serum ferritin (SF) target level of 50 microg/L, a two-treatment-arms, randomized trial was conducted in which 38 newly diagnosed patients homozygous for C282Y were randomly assigned in a 1:1 ratio to undergo either Erythrocytapheresis or phlebotomy. A 50% decrease in the number of treatment procedures for Erythrocytapheresis compared to phlebotomy was chosen as the relevant difference to detect. RESULTS: Univariate analysis showed a significantly lower mean number of treatment procedures in the Erythrocytapheresis group (9 vs. 27; ratio, 0.33; 95% confidence interval [CI], 0.25-0.45; Mann-Whitney p < 0.001). After adjustments for the two important influential factors initial SF level and body weight, the reduction ratio was still significant (0.43; 95% CI, 0.35-0.52; p < 0.001). Cost analysis showed no significant difference in treatment costs between both procedures. The costs resulting from productivity loss were significantly lower for the Erythrocytapheresis group. CONCLUSION: Erythrocytapheresis is highly effective treatment to reduce iron overload and from a societal perspective might potentially also be a cost-saving therapy.
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Erythrocytapheresis for hereditary haemochromatosis
Nederlands tijdschrift voor geneeskunde, 2012Co-Authors: Eva Rombout-sestrienkova, Mirian C H Janssen, C.t. Van Deursen, M.g.j. Van Kraaij, P.w. De Leeuw, Ger H KoekAbstract:With a prevalence of 0.4%, hereditary haemochromatosis is the most common autosomal-recessive genetic disease in Northern Europe. Hereditary haemochromatosis is characterized by an increase in the absorption of iron. This consequently leads to the excessive deposition of iron in tissues and organs with resultant functional impairment. Early-stage treatment can prevent complications resulting from the accumulation of iron. The standard treatment for hereditary haemochromatosis is phlebotomy, whereby 500 ml of whole blood is removed once a week until serum ferritin levels of 50-100 mug/l are achieved. After this, the patient must undergo lifelong maintenance treatment consisting of 3-6 phlebotomies per year on average. An alternative treatment is Erythrocytapheresis, the selective removal of erythrocytes by way of apheresis. This procedure makes it is possible to collect more erythrocytes than during phlebotomy, resulting in significantly fewer treatment sessions being needed for the initial removal of overabundant iron.
Agnès Glacet-bernard - One of the best experts on this subject based on the ideXlab platform.
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Hemodilution therapy using automated Erythrocytapheresis in central retinal vein occlusion: results of a multicenter randomized controlled study
Graefe's Archive for Clinical and Experimental Ophthalmology, 2011Co-Authors: Agnès Glacet-bernard, Marielle Atassi, Christine Fardeau, Jean-paul Romanet, Matthieu Tonini, John Conrath, Philippe Denis, Martine Mauget-faÿsse, Gabriel Coscas, Gisèle SoubraneAbstract:Background Central retinal vein occlusion (CRVO) leads to poor visual outcome in most eyes. Abnormal hemorheology was suspected to play a major role in its pathogenesis. CRVO treatment is still a matter of debate but several studies have pointed out the efficacy of isovolumic hemodilution. The aim of this study was to assess the feasibility and efficacy of hemodilution using automated Erythrocytapheresis in recent-onset CRVO. Methods In this prospective randomized controlled multicenter study, 61 consecutive CRVO patients were enrolled when they met the following criteria: CRVO lasting for 3 weeks or less, visual acuity ranging from 20/200 to 20/32, age between 18 and 85 years, no diabetes, no uncontrolled systemic hypertension, no antiplatelet or anticoagulant therapy, hematocrit higher than 38%, and signed informed consent. Patients were randomly assigned to the hemodilution group ( n = 31) or to the control group ( n = 30). Hemodilution therapy consisted of one session of Erythrocytapheresis on outpatient basis, followed by additional session(s) for 6 weeks if needed. Target hematocrit was 35%. Follow-up was 12 months. Results No statistical differences in age, associated risk factors, or CRVO characteristics were observed at baseline between both groups. Mean visual acuity was equivalent to 20/80 in the hemodilution group and to 20/63 in the control group (non-significant difference). In the treated group, mean number of hemodilution sessions was 3.3 (range, 1 to 6), and no major side-effects occurred. At the 12-month follow-up visit, 64.5% of the hemodilution group had visual acuity of 20/40 or better compared to 40% of the control group ( p = .048). Visual change was a gain of 1.7 ETDRS line in the hemodilution group versus a loss of 2.3 lines in the control group ( p = .007). There was less conversion into an ischemic form in the hemodilution group (11%) than in the control group (50%, p = .004). Mean final retinal thickness was 289 μm in the hemodilution group versus 401 μm in the control group ( p = .068). Conclusions This multicenter controlled randomized study demonstrated that automated Erythrocytapheresis is a safe and effective tool for performing hemodilution and confirmed that hemodilution therapy can improve the final prognosis of CRVO when applied in the early phase of the disease.
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Hemodilution therapy using automated Erythrocytapheresis in central retinal vein occlusion: results of a multicenter randomized controlled study
Graefe's Archive for Clinical and Experimental Ophthalmology, 2011Co-Authors: Agnès Glacet-bernard, Marielle Atassi, Christine Fardeau, Jean-paul Romanet, Matthieu Tonini, John Conrath, Philippe Denis, Martine Mauget-faÿsse, Gabriel Coscas, Gisèle SoubraneAbstract:Background Central retinal vein occlusion (CRVO) leads to poor visual outcome in most eyes. Abnormal hemorheology was suspected to play a major role in its pathogenesis. CRVO treatment is still a matter of debate but several studies have pointed out the efficacy of isovolumic hemodilution. The aim of this study was to assess the feasibility and efficacy of hemodilution using automated Erythrocytapheresis in recent-onset CRVO. Methods In this prospective randomized controlled multicenter study, 61 consecutive CRVO patients were enrolled when they met the following criteria: CRVO lasting for 3 weeks or less, visual acuity ranging from 20/200 to 20/32, age between 18 and 85 years, no diabetes, no uncontrolled systemic hypertension, no antiplatelet or anticoagulant therapy, hematocrit higher than 38%, and signed informed consent. Patients were randomly assigned to the hemodilution group ( n = 31) or to the control group ( n = 30). Hemodilution therapy consisted of one session of Erythrocytapheresis on outpatient basis, followed by additional session(s) for 6 weeks if needed. Target hematocrit was 35%. Follow-up was 12 months. Results No statistical differences in age, associated risk factors, or CRVO characteristics were observed at baseline between both groups. Mean visual acuity was equivalent to 20/80 in the hemodilution group and to 20/63 in the control group (non-significant difference). In the treated group, mean number of hemodilution sessions was 3.3 (range, 1 to 6), and no major side-effects occurred. At the 12-month follow-up visit, 64.5% of the hemodilution group had visual acuity of 20/40 or better compared to 40% of the control group ( p = .048). Visual change was a gain of 1.7 ETDRS line in the hemodilution group versus a loss of 2.3 lines in the control group ( p = .007). There was less conversion into an ischemic form in the hemodilution group (11%) than in the control group (50%, p = .004). Mean final retinal thickness was 289 μm in the hemodilution group versus 401 μm in the control group ( p = .068). Conclusions This multicenter controlled randomized study demonstrated that automated Erythrocytapheresis is a safe and effective tool for performing hemodilution and confirmed that hemodilution therapy can improve the final prognosis of CRVO when applied in the early phase of the disease.
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Hemodilution therapy using automated Erythrocytapheresis in central retinal vein occlusion: results of a multicenter randomized controlled study.
Graefe's Archive for Clinical and Experimental Ophthalmology, 2010Co-Authors: Agnès Glacet-bernard, Marielle Atassi, Christine Fardeau, Jean-paul Romanet, Matthieu Tonini, John Conrath, Philippe Denis, Martine Mauget-faÿsse, Gabriel Coscas, Gisèle SoubraneAbstract:Background Central retinal vein occlusion (CRVO) leads to poor visual outcome in most eyes. Abnormal hemorheology was suspected to play a major role in its pathogenesis. CRVO treatment is still a matter of debate but several studies have pointed out the efficacy of isovolumic hemodilution. The aim of this study was to assess the feasibility and efficacy of hemodilution using automated Erythrocytapheresis in recent-onset CRVO.
Salvatore Bertolone - One of the best experts on this subject based on the ideXlab platform.
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long term Erythrocytapheresis is associated with reduced liver iron concentration in sickle cell disease
Journal of Pediatric Hematology Oncology, 2016Co-Authors: Scott N. Myers, Salvatore Bertolone, Ryan Eid, John Myers, Arun Ranjan Panigrahi, Jennifer Mullinax, Ashok RajAbstract:BACKGROUND Erythrocytapheresis procedures are increasingly used in sickle cell disease. Serum ferritin and noninvasive magnetic resonance imaging measurements of liver iron concentration (LIC) are frequently used to monitor iron overload secondary to hypertransfusion. There is a paucity of data describing the impact of long-term Erythrocytapheresis (LTE) on LIC. MATERIALS AND METHODS We measured magnetic resonance imaging liver and cardiac iron on LTE subjects and stratified them into 2 groups: higher LIC (>3 mg/g) and lower LIC (<3 mg/g). χ(2) and t test were used to test for differences between the 2 groups. Logistic regression and generalized linear mixed-effects models were used to test what impacted LIC. RESULTS None of 29 sickle cell disease subjects maintained on LTE had high cardiac iron concentration. LIC was associated with serum ferritin (r=0.697, P<0.001) but was not associated with the total number of LTE procedures (r=-0.088, P=0.656) or total number of simple transfusions (r=0.316, P=0.108). The total number of LTE procedures was not associated with serum ferritin (r=0.040, P=0.838), the total number of simple transfusions (r=-0.258, P=0.184), or LIC group (r=-0.111, P=0.566). CONCLUSION There was no significant correlation between duration of LTE maintenance and LIC.
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Long-term Erythrocytapheresis Is Associated With Reduced Liver Iron Concentration in Sickle Cell Disease.
Journal of Pediatric Hematology Oncology, 2016Co-Authors: Scott N. Myers, Salvatore Bertolone, Ryan Eid, John Myers, Arun Ranjan Panigrahi, Jennifer Mullinax, Ashok RajAbstract:BACKGROUND Erythrocytapheresis procedures are increasingly used in sickle cell disease. Serum ferritin and noninvasive magnetic resonance imaging measurements of liver iron concentration (LIC) are frequently used to monitor iron overload secondary to hypertransfusion. There is a paucity of data describing the impact of long-term Erythrocytapheresis (LTE) on LIC. MATERIALS AND METHODS We measured magnetic resonance imaging liver and cardiac iron on LTE subjects and stratified them into 2 groups: higher LIC (>3 mg/g) and lower LIC (
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Utility of impedance cardiography for the detection of hemodynamic changes in stable patients with sickle cell disease
Journal of Pediatric Hematology Oncology, 2012Co-Authors: Bibhuti B. Das, Maiying Kong, Ashok Raj, Michael R. Recto, Salvatore BertoloneAbstract:OBJECTIVES The study sought to assess the potential utility of impedance cardiography (ICG) to detect hemodynamic changes after Erythrocytapheresis in stable children with sickle cell disease (SCD). METHODS We prospectively monitored cardiac index, systemic vascular resistance index, heart rate, and blood pressure using ICG before and after Erythrocytapheresis in 26 stable children with SCD. Echocardiography was carried out in all patients to evaluate left ventricular systolic function. Hemoglobin (Hb), sickle cell hemoglobin (HbS), and ferritin levels were also measured. RESULTS Of a total of 78 Erythrocytapheresis procedures in 26 children with SCD, 22 (28.2%) had hypotensive episodes defined as a decrease in systolic, diastolic, or mean blood pressure by 10 mmHg. Risk factors for developing hypotension during Erythrocytapheresis were identified with logistic regression analysis: lower-body surface area and decrease in cardiac index. In contrast, age, prepheresis Hb and HbS, serum ferritin levels, and left ventricular function at baseline were not associated with hypotension. CONCLUSIONS This study demonstrates the feasibility of the ICG technique to detect the hemodynamic changes in children with SCD after an Erythrocytapheresis procedure.
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Antibody development in pediatric sickle cell patients undergoing Erythrocytapheresis.
Pediatric Blood & Cancer, 2010Co-Authors: Gwendolyn J Godfrey, Salvatore Bertolone, Maiying Kong, William Lockwood, Ashok RajAbstract:Background Erythrocytapheresis, or red blood cell exchange transfusion (RBCX), with donor red blood cell (RBC) units is now increasingly used in the treatment of acute and chronic complications of sickle cell disease (SCD). As in all transfusions, RCBX carries a risk of immunization against foreign antigen on transfused cells. However, by selecting donor units with RBC phenotypes similar to the patient, the risk of allo- and autoimmunization can be reduced. Procedure The formation of RBC alloantibodies and/or autoantibodies in 32 multitransfused pediatric SCD patients undergoing monthly RBCX over a 11-year period (12/1998 to 12/2009) was evaluated utilizing a retrospective patient chart review at Kosair Children's Hospital, Louisville, Kentucky. Results After starting C, E, K antigen-matched RBCX, the rate of clinically significant allo-immunization decreased from 0.189/100 to 0.053/100 U, with a relative risk of 27.9%. Likewise, the rate of autoimmunization decreased from 0.063/100 to 0.035/100 U, with a relative risk of 55.9%. Conclusion After controlling for clinically insignificant antibodies, our auto- and alloimmunization rate was much less than previously reported values. In addition, the incidence of clinically significant allo- and autoimmunization decreased in our patient population after starting minor antigen-matched RBCX. These results suggest that by matching selected RBC phenotypes, there may be an association in the risk of allo- and autoimmunization of multi-transfused SCD patients. Pediatr Blood Cancer. 2010;55:1134–1137. © 2010 Wiley-Liss, Inc.
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Hemodynamic Monitoring during Erythrocytapheresis in Sickle Cell Disease Patients Using Impedance Cardiography
Blood, 2008Co-Authors: Bibhuti B. Das, Samyuktha Kemparajurs, Salvatore Bertolone, Maiying Kong, Ashok RajAbstract:BACKGROUND: Erythrocytapheresis procedures are increasingly used in the treatment of severe acute complications of sickle cell disease (SCD). Some patients who undergo Erythrocytapheresis experience dizziness during or after the procedure. There is no data in literature for hemodynamic monitoring of cardiac index (CI) and systemic vascular resistance index (SVRI) during Erythrocytapheresis. We have previously shown that impedance cardiography (ICG) and invasive measures of CI by thermodilution techniques are significantly correlated in children. OBJECTIVES: We wanted to evaluate changes in CI and SVRI using ICG and to correlate these with systolic blood pressure (SBP) measurements in SCD patients while undergoing Erythrocytapheresis. METHODS: Thirty one SCD patients who had 70 consecutive Erythrocytapheresis procedures were included in this IRB approved study. ICG measurements (CI, SVRI, and blood pressure) were taken pre-Erythrocytapheresis, 30 minutes after initiation of the procedure, one hour into Erythrocytapheresis, and at the end of the procedure. ICG measurements were repeated in symptomatic patients before and after intervention with fluid administration. RESULTS: We evaluated hemodynamic data on 31 SCD patients, average age 12.8±5.2 years and 56% are female. SBP decreased below the base line during 33 procedures and increased above the baseline during 37 procedures. Among patients who had a decrease in SBP, 28/33 (84%) had decreased CI. Among them 22/28 (78%) had increased SVRI. Among patients who had an increase in SBP, 5/37 (14%) had increased CI. Among them 4/5 (80%) also had increased SVRI. Using paired t test, the changes in CI (P CONCLUSIONS: In summary, the hemodynamic changes during Erythrocytapheresis are probably multifactorial in origin and may include a combination of SVRI and CI changes. Confirmation of these findings may allow early intervention in some patients and minimize the risk of developing overt clinical symptoms during Erythrocytapheresis.