scholarly journals Combination of Exchange Transfusion Treatment and Hydroxyurea Cause Beneficial Changes to Laboratory Parameters and Clinical Outcome in Patients with Sickle Cell Disease/β Thalassemia Compared with Hydroxyurea or Exchange Transfusion Alone

2016 ◽  
Vol 2 (4) ◽  
Author(s):  
Sophia Delicou
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Outcome ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Cell Disease ◽  
Laboratory Parameters

Manual Chronic Partial Exchange In Children and Adolescents with Sickle Cell Disease: Impact on Clinical Outcome and Iron Overload

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4821-4821
Author(s):  
Michel Aloni ◽  
Alina Ferster ◽  
Phu-Quoc Le ◽  
Catherine Heijmans ◽  
Sophie Huybrechts ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Outcome ◽  
Iron Overload ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Red Cell ◽  
Cell Disease ◽  
Hb S ◽  
The Mean

Abstract Abstract 4821 Background: Red cell exchange transfusion is frequently used in the management of patients with sickle cell disease either electively or therapeutically to maintain an hemoglobin S (Hb S) level < 30–50%. This target is often difficult to maintain. In order to assess the effects of chronic partial exchange transfusion (CPET) a) on level of Hb and Hb S, b) on iron overload c) the need for chelation, d) on risk of long term adverse events and e) clinical outcome, we analyzed the data of sickle cell disease patients treated by long term CPET in our center. Methods/subjects: In the cohort of 163 SCD patients followed at University Children's Hospital at Brussels (Belgium), 10 benefit from CPET. Main reasons for CPET were neurologic disease (4), frequent ACS (3), previous severe hepatic cholestasis (2), leg ulcer (1) and pulmonary hypertension (1). The median age at start of treatment was 13 years (range 4 –19). These patients (6 males and 4 females) account for 248 exchanges during a median follow-up of 20 months (range 6– 36). These exchanges are until now performed manually and the volume exchanged is calculated taking into account the Hb level and the last HbS percentage. It is usually between 30 and 40 ml/kg BW. Except if severe anemia occurs, the goal of these exchanges is to keep a constant hematocrit level. All patients had a full red cell phenotype performed and received blood matched for ABO, Rhesus, Kell and Duffy antigens systems. The estimation of iron balance (iron intake- iron removed) was calculated yearly. Results: The pre-exchange Hb value was 9.5 g/dl (median; range: 7.7–10.9 g/dl) and the mean post value was 9.4/dl (range: 8.4– 11.1 g/dl). These values are not statistically different (p> 0.05). The majority of patients (9/10) are reached an HbS < 50% when measured 3–5 weeks after PET (just before the next procedure) with a median HbS value of 40% (range: 30–54). At start of CPET program, the median ferritin level was 439 ng/ml (range: 80 – 1704). Five patients had already a ferritin > 500 ng/mL due to numerous previous transfusions. At last evaluation, the median ferritin did not change significantly and was 531 ng/ml (range 84– 3840). The two patients with ferritin higher than 1000 ng/ml start chelation with good result for one. One The mean annual net RBC load were 1.72 ml RBC/kg/yr provided approximately 1.85 mg of iron/kg/yr. Individual data are given in table 1. CPET-treated patients exchanged showed clinical improvement with disappearance of SCD crisis and related complications. The procedure was well tolerated by most patients, and adverse effects were limited to mild hypotension (3/10). No autoimmune hemolysis or allo-immunisation was documented in this cohort. All children remained negative for HIV and hepatitis C virus infections. Conclusion: Manual CPET seems to be safe to prevent middle-term iron overload and the need of elation therapy in most of patients. CPET can therefore be recommended for SCD patients who required decreased in Hb S levels either prophylactically or therapeutically. Manual are safe, effective and easy to use when mechanized exchanges are not possible for technical reasons. Disclosures: No relevant conflicts of interest to declare.

Non-crisis related pain occurs in adult patients with sickle cell disease despite chronic red blood cell exchange transfusion therapy

2021 ◽  
pp. 103304
Author(s):  
Susanna A. Curtis ◽  
Balbuena-Merle Raisa ◽  
John D. Roberts ◽  
Jeanne E. Hendrickson ◽  
Joanna Starrels ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Exchange Transfusion ◽  
Adult Patients ◽  
Cell Disease ◽  
Transfusion Therapy

Clinical outcome and laboratory parameters in sickle cell anemia patients of pediatric age group post hydroxyurea therapy (Preprint)

2021 ◽  
Author(s):  
Nihar Gupta
Keyword(s):  
Clinical Outcome ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Age Group ◽  
Pediatric Age ◽  
Cell Disease ◽  
Laboratory Parameters ◽  
Pediatric Age Group ◽  
Group Post ◽  
Hydroxyurea Therapy

BACKGROUND Sickle cell anemia or SCA is a homozygous condition of Sickle cell disease or SCD, in which patients presents with a chronic and progressive condition which is characterised by hemolytic anemia, recurrent vaso-occlusive events, along with complications like organ dysfunction. Hydroxyurea has emerged as a break-through in treatment of Sickle cell anemia. Currently it is the only FDA approved drug which has shown disease modifying results. OBJECTIVE To determine the clinical outcome and laboratory parameters in SCA patients of pediatric age group post-hydroxyurea therapy. METHODS A total of 30 patients who were diagnosed as Sickle cell anemia(SCA) patients in Sickle cell anemia OPD of Pediatric department were included in the study. RESULTS Hydroxyurea therapy is expected to increase HbF% levels and improve the clinical outcome and laboratory parameters in sickle cell anemia patients of pediatric age group. CONCLUSIONS Hydroxyurea use increases HbF%, decrease painful crises, blood transfusion, and days of hospitalisation.

scholarly journals How I treat and manage strokes in sickle cell disease

Blood ◽  
2015 ◽  
Vol 125 (22) ◽  
pp. 3401-3410 ◽  
Author(s):  
Adetola A. Kassim ◽  
Najibah A. Galadanci ◽  
Sumit Pruthi ◽  
Michael R. DeBaun
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Management Strategies ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Hematopoietic Stem ◽  
Neuro Imaging

Abstract Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ∼11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by a multidisciplinary team (a hematologist, neurologist, neuroradiologist, and transfusion medicine specialist); prompt neuro-imaging and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion) is recommended if the hemoglobin is >4 gm/dL and <10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in SCD.

Alterations of Hemoglobin Fractionation in a Sickle Cell Disease Patient on Voxelotor Therapy

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S100-S101
Author(s):  
S S Karimi ◽  
H Ni ◽  
L L Hsu
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Recent Literature ◽  
Disease Patient ◽  
Oxygen Affinity ◽  
Treatment Modalities ◽  
Cell Disease ◽  
Low Oxygen ◽  
Hydroxyurea Treatment

Abstract Introduction/Objective Voxelotor is a molecule that allosterically binds to the alpha-chain of hemoglobin, resulting in increased oxygen affinity. This allosteric inhibition leads to prevention of hemoglobin polymerization and sickling of red blood cells in response to low oxygen tension. Voxelotor has been used to treat patients with Sickle Cell Disease (SCD) and recent literature indicates it may contribute to complex hemoglobin fractionation (HF) elution patterns. We report a novel case of a SCD patient on concurrent Hydroxyurea, Voxelotor and chronic RBC exchange transfusion treatment and discuss the implications of these three treatment modalities on HF and monitoring of SCD. Methods A 17-year-old female with SCD complicated by frequent vaso-occlusive crisis, and avascular necrosis managed with chronic RBC exchange and Hydroxyurea. Her HF prior to initiation of Voxelotor treatment showed 3.2% HbA2, 51% HbA, 6.0% HbF, and 41% HbS. Voxelotor therapy was initiated at 1500mg/day and HF was performed 10 days later. Whole blood was collected and subjected to High Performance Liquid Chromatography (HPLC) with reflex to RBC solubility and Capillary Electrophoresis. Results HF performed post-Voxelotor therapy revealed positive sickle solubility with a complex pattern of 2.7% HbA2, 49.2% HbA, 5.3% HbF, 15.7% HbS, 0% HbC, and two additional peaks of a 6.3% peak in the window-D region (retention time of 4.34) and 20.8% of an atypical Hb peak pattern (at the retentuin time of 4.18). The results reflected a complex HF of a HbSS patient on concurrent chronic RBC exchange transfusion, hydroxyurea therapy, and Voxelotor treatment. Post Voxelotor-therapy HF revealed a reduction in HbS from 41% to 15.7% with the emergence of two additional peaks. Chronic RBC exchange transfusion and Hydroxyurea treatment account for the observed fractionation of HbA and HbF, respectively. Based on recent literature, we attribute the emergence of the two additional peaks to Voxelotor therapy. All three therapies led to reduction in HbS. Conclusion Routine HF serves as an essential modality in diagnosis and monitoring of SCD. Voxelotor treatment alters the HF profile and may cause difficulty for interpretation. With the emergence of novel therapies, it is imperative for clinicians to provide medication information to clinical laboratories and pathologists to be fully aware of the effects of current treatments to correctly interpret and monitor SCD.

scholarly journals Identification of Clinical and Laboratory Parameters Associated with the Development of Acute Chest Syndrome during Vaso-Occlusive Episodes in Children with Sickle Cell Disease: A Preliminary Step before Assessing Specific and Early Treatment Strategies

2019 ◽  
Vol 8 (11) ◽  
pp. 1839
Author(s):  
Madhi ◽  
Kamdem ◽  
Jung ◽  
Carlier-Gonod ◽  
Biscardi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pain Score ◽  
Sickle Cell ◽  
Multivariate Model ◽  
Red Blood Cell Transfusion ◽  
Predictive Score ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Laboratory Parameters ◽  
Increased Risk

This prospective observational study sought to ascertain clinical and laboratory parameters associated with the development of acute chest syndrome (ACS) during vaso-occlusive episodes (VOE) in children with sickle cell disease (SCD). It was performed at the pediatric department of the university Intercommunal Créteil hospital. All children with SCD (all sickle genotypes) consecutively admitted from November 2013 to December 2016 for painful VOEs and no evidence of ACS were included. Clinical and laboratory parameters collected at admission and within 48 h after admission were compared for children in whom ACS developed or not. Variables that were statistically significant on univariate analysis or considered to be clinically relevant were included in a multivariable model to ascertain the risk factors associated with the development of ACS during a VOE. The variables retained in the multivariate model were used to construct a predictive score for ACS. For each included child and during the study period, only data from the first VOE and/or the first ACS were analyzed. Among 191 hospitalizations for painful VOEs, for 176 children with SCD, ACS developed in 35 during hospitalization. Mean hospital stay was longer for children with ACS versus VOEs alone (7.6 (±2.3) vs. 3.3 (±1.8) days, p < 0.0001), and all children with ACS versus 28/156 (17.9%) with VOEs alone received red blood cell transfusion (p < 0.0001). The multivariate model retained pain score (≥9/10), pain localization (abdominal or spinal pain or involving more than two limbs), and high reticulocyte (≥260 × 109/L) and neutrophil (>10 × 109/L) counts, at admission, as independently associated with ACS development. The area under the receiver operating characteristic curve for the ACS predictive score was 0.82 (95% CI: 0.74–0.89), and the negative predictive value was 97.7%. The evolution profiles during the first 48 h differed between children with ACS and VOEs alone, with a more rapid decline of pain score and leucocytosis in children with VOEs. Clinical and laboratory measurements at admission may be simple parameters to identify children with increased risk of ACS development during VOEs and to facilitate early diagnosis of this respiratory complication. Also, the persistent elevation of leukocyte count on day 2 may be considered a sign of evolving ACS.

Red Blood Cell Exchange Transfusion, An Underutilized Effective and Reliable Therapeutic Modality in Sickle Cell Disease, Reduces Number of Admissions and Length of Hospital Stay

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4822-4822
Author(s):  
Aref Agheli ◽  
Kirshma Khemani ◽  
Madhumati Kalavar ◽  
William Steier ◽  
Zili He
Keyword(s):  
Sickle Cell Disease ◽  
Hospital Stay ◽  
Sickle Cell ◽  
Blood Viscosity ◽  
Exchange Transfusion ◽  
Hemoglobin Level ◽  
Therapeutic Modality ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
One Year

Abstract Background: The pathophysiology of sickle cell disease (SCD) is based on increased blood viscosity due to abnormal red blood cells (RBCs), which causes SCD complications, such as chronic hemolytic anemia, vaso-occlusive crisis with tissue hypoxemia, and organ dysfunction. Effective treatment of Sickle Cell Anemia is to reduce the blood concentration of Hemoglobin S (Hb S) RBCs. Exchange transfusion (ET) remains an effective but possibly underutilized therapy for the management of various acute and chronic complications of SCD such as acute chest syndrome, thromboembolic stroke, splenic and hepatic infarction, right upper quadrant syndrome, multi-organ failure syndrome, or in preparation for surgery by reducing HbS to less than 30%. RBC ET quickly replaces abnormal RBCs with normal RBCs, thus improving oxygen transport while reducing overall blood viscosity. Methodology: To determine the effectiveness of ET in SCD in reducing the total number of admissions and total in-hospital Length of Stay (LOS) in patients, admitted with any of acute complications of SCD, we retrospectively reviewed the medical records of 38 patients between June 15, 2007 and June 15, 2008. The eligibility criteria were age above 18 years old and admission to the hospital for any SCD complication. Nineteen patients had ET with Hb A containing RBCs, generally with an average packed RBC exchange volume of 70–80 ml/kg patient’s weight. Nineteen patients were treated with conventional managements. Three patients were excluded from the ET group because of prolonged LOS due to non-SCD- related complications. Four patients in the non-Exchange group signed against medical advice on the first day of admission and were excluded from analysis. Results: Sixteen (42.1%) patients were male and 22 (57.9%) patients were female. Their ages ranged from 19 to 67 years old, mean (SD) 30.2 (10.8). Of eligible patients, 19 (50%) patients received at least one therapeutic ET during the one year period of the study. In an independent-Samples T test analysis, the mean (SD) LOS were 7.5 (0.6) and 4.2 (0.6) days for the groups without ET and with ET respectively (95% CI = −5.2 to 1.5, p=.0011) (Figure 1). In this small studied group, this resulted average 3.3 days shorter in-hospital stay in ET group, could have saved 62 in-hospital days in the group who received conventional treatments. This number could have been easily much greater, since our hospital’s electronically stored data revealed that during year 2007, there had been 278 SCD admissions in all age groups. There was no mortality in the ET group, nor were any transfusion-related complications reported. In another analysis of one year follow up data, the number of admissions for the patients who never received ET ranged from 1 to 14, mean (SD) 1.7 (2.3) times in year 2007, while in patients who received at least one ET, the number of following admissions ranged from 0 to 2, mean (SD) 0.7 (1.3) times during the next year (95% CI = 0.16 to 1.7, p= .020). Hemoglobin level of patients in conventional treatment group on the day of discharge ranged from 6.5 to 10.7, mean (SD) 8.9 (1.9) and in ET group it ranged from 8.4 to 12.4, mean (SD) 10.2 (1.2) gr/dl (p= .045). Conclusion: Patients with SCD are frequently admitted to hospital for vaso-occlusive crisis and other complications. Exchange transfusion is a reliable, safe, and effective therapeutic modality in SCD patients, in particular during a catastrophic event. ET can significantly reduce the number of hospital admissions and in-hospital stay days in these patients. In addition, patients managed with ET have a better hemoglobin level on discharge. Figure Figure

Are There True Non-Responders to Hydroxyurea in Sickle Cell Disease? a Multiparameter Analysis

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4073-4073 ◽  
Author(s):  
Arati Rani Chand ◽  
Hongyan Xu ◽  
Leigh G Wells ◽  
Betsy Clair ◽  
Cindy Neunert ◽  
...  
Keyword(s):  
Regression Analysis ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Response Rate ◽  
Treatment Time ◽  
Conflicts Of Interest ◽  
Statistical Significance ◽  
Average Dose ◽  
Cell Disease ◽  
Laboratory Parameters

Abstract Hydroxyurea (HU) is the only FDA approved anti-switching agent for the management of sickle cell disease (SCD). The non-response rate to HU (inadequate increase in fetal hemoglobin (HbF)) has been reported to be as high as 30%. However, the role of patient non-compliance as a cause of sub-optimal response to HU has not been studied. Establishing the rate of non-response to HU despite adequate dose and compliance would help in ascertaining patients who might benefit from alternate treatment strategies in SCD. The primary objective of this study was to differentiate between non-compliance and lack of response in patients using laboratory parameters other than HbF. We conducted a retrospective review of 137 adult SCD patients from GRU's Sickle Cell Center that were reportedly taking HU for ≥ 6 months. Data included weight, dose, HbF, Hb, RBC, RDW, retic, MCV, MCH, WBC, ANC, platelets, bilirubin, prior to initiation of HU therapy and at the time of maximal HbF response. Dose of HU/Kg required to achieve that response and time to response was calculated. We defined response as an absolute HbF value of ≥20% or a ≥5% increase in HbF from baseline. The anticipated direction of change in laboratory parameters indicative of compliance was an increase in MCV, MCH, Hb, PCV, and a decrease in RDW, retic count, WBC, neutrophils, platelets, and bilirubin. Patients without a change in HbF but with response from the additional parameters were classified as inadequate-responders. Patients without response in HbF as well as additional parameters were classified as non-compliant. We performed a regression analysis to study the effect of dose and patient age on the response (change in HbF). To model dosage, a new variable called HU exposure was calculated as the product of dose (mg/kg/day) and treatment time (days). The HU exposure and age was used as the predictors for change in HBF. Our results showed that of the 137 patients, 82(59.9%) were responders to HU (mean dose of 18.8mg/kg) with an expected increase in HbF and 36(26.3%) were non-compliant (mean dose of 18.48mg/kg) based on the fact that the aforementioned laboratory values did not change significantly. Only 19(13.9%) were non-responders (mean dose of 16.84 mg/kg) based on a lack of HbF response despite other laboratory parameters being indicative of compliance with HU. Out of the 19 patients who were non-responders only 2 patients were on HU doses more than 20mg/kg. In the overall sample, we found that both age and HU exposure were positively associated with HbF change, which was highly statistically significant (p=2.55E-08 and 7.03E-10, respectively). When we performed regression analysis in the responders, non-responders and non-compliant groups separately we found that in the responder group both age and HU exposure were positively associated with HbF change, with high statistical significance. In the non-responder group however, age and HU exposure were not statistically significant. Patients with SCD on HU who fail to show an adequate increase in HbF are more likely to be non-compliant with medication than actually being truly resistant to it. We have shown that laboratory measurements beside HbF help make the distinction between true HU non-responders and non-compliant patients. We believe a non-response rate of 13.9% may still be too high based on the fact that the average dose of HU was 16.97mg/kg, barely over the recommended starting dose of 15 mg/kg/day. These data suggest that among adult patients on HU, the rates of the non-responders are very low when given adequate doses of HU for sufficient periods of time. Disclosures No relevant conflicts of interest to declare.

Clinical Benefit and Costs’ Evaluation of Erythocytapheresis Compared to Manual Exchange Transfusion for Children with Sickle Cell Disease: A Single Center Experience

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4091-4091
Author(s):  
Laurence Dedeken ◽  
Phu Quoc Lê ◽  
Laurence Rozen ◽  
Hanane El Kenz ◽  
Sophie Huybrechts ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Median Duration ◽  
Exchange Transfusion ◽  
Biological Data ◽  
Acute Chest Syndrome ◽  
Continuous Control ◽  
Cell Disease ◽  
Previously Treated

Abstract Chronic transfusion in sickle cell disease (SCD) remains the gold standard therapy for primary and secondary stroke prevention and is indicated for patients with recurrent severe vaso occlusive crisis (VOC) or acute chest syndrome (ACS). Automatized apheresis (AA) has several advantages compared to manual exchange transfusion (MET): shorter procedure, continuous control of fluid balance, etc. The aim of our study was to assess the safety and efficacy of AA in SCD patients previously treated with MET at Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium and to evaluate the change of the costs related to transfusion and chelation overtime. From January 2012, the AA program for SCD patients started in our institution. Patients on chronic transfusion program (CTP) and previously treated with MET were eligible to switch to AA if sufficient peripheral venous access to allow AA without the use of central venous line and if weight ≥ 30kg. On CTP, target HbS was <30% in case of stroke risk and <50% for other indications. Data on biological values, duration of the procedure, intervals between procedures as well as adverse events were recorded for the last 6 months on MET and compared to the data on AA. The overall costs of last year on MET, 1st year and 2nd year on AA were analysed. The cost of packed red blood cell (RBC), one-day care facility, apheresis kit and chelation were recorded. For patients on AA for less than 2 years, costs for the 2nd year were extrapolated taking into account the data from the 6 previous months. Data were collected for this analysis until June 2014. Friedman test was used to compare treatment across years and Dunn's Multiple Comparison Test to compare each year of treatment among them. Ten patients switched from MET to AA at a median age of 11.8 years (range, 9.6-16.8y). The median duration of MET before start of AA was 1.9 years (range 0.5-4.4y). The median duration of AA was 1.7 years (range 1-2.4y). Four patients are on AA for > 2 years, 4 ≥ 20 months and 2 > than 12 months. Indications for CTP were overt stroke (2), pulmonary hypertension (2), recurrent VOC/ACS (5) and poorly tolerated severe anemia (1). All patients remained stable without any SCD related event, except one child who presented seizures without evidence of new stroke and for whom anticonvulsivant therapy was resumed. HbS level remained in the target values for all patients despite a slightly but significantly higher value on AA. The ferritin level and the duration of the procedure decreased significantly (Table 1). The 2 patients on iron chelation stopped it after 10 and 1 AA procedures. Interval between 2 AA was significantly longer than on MET (P<0.0001). On 181 procedures, 9 adverse events (4.9%) required medical intervention: transient hypotension (1), symptomatic hypocalcemia (2), transient headache (2), fever (1), nausea-vomiting (1) and abdominal pain (2). On AA, the requirement of packed RBC was significantly higher than on MET. During the 1st year, costs of AA were significantly higher than MET (132937€ vs.107560€; P=0.01). Nevertheless, during the 2nd year of treatment, the costs of AA were not significantly different from those on MET (102965€ vs. 107560€). Indeed chelation could be stopped in patients previously treated. AA is useful and safe for SCD patients requiring exchange transfusion program. It is less time consuming for nurses and patients, improves iron overload and interval between 2 procedures is significantly reduced. Despite higher costs related to the increase packed RBC requirement, the costs of AA and MET in the Belgian Health Care System are the same as chelation could be stopped in previously treated patients. Abstract 4091. Table 1.Changes in age, weight, biological data and procedure parameters on MET and on AA On METOn AAP value 1st year 2nd yearMedianRangeMedianRangeMedianRangeMedian age (years)11,89,7-16,812,810,8-17,713,311,8-18,6<0,0001Median weight (kg)45,530,4-66,349,933,8-72,05336,1-76,0<0,0001Median height (cm)153,5138-178161143-180165145-182<0,0001Hb (g/L)9,958,6-10,89,958,8-10,6109,2-11,7NSHb S (%)33,525-424028,5-424631-480,0002Ferritin (µg/L)666182-151225552-8111489-622<0,001Duration of procedure (min)245195-36087,375,5-1269164-1540,0002Interval between procedures (d)2821-293428-35,54228-42<0,0001Packed RBC requirement (ml/kg)18,315,1-2032,227,4-36,13026,8-36<0,0001Packed RBC requirement (unit)39,515-796749-12065,538-137<0,0001 Disclosures No relevant conflicts of interest to declare.

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