Manual Exchange Transfusion Research Articles

Manual Exchange Transfusion Role in the Management of a Pregnancy Compromised by Sickle Cell Disease in a Tertiary Hospital in Burkina Faso: A Case Report

Sickle cell disease in pregnant women can lead to potentially serious complications for both the mother and fetus. Its management remains a major public health challenge, particularly in resource-limited regions. Here, we report the management of a case at the Regional University Hospital (RUH) of Ouahigouya. A 30-year-old patient with 2 pregnancies, 1 miscarriage following a vaso-occlusive crisis. The current pregnancy was monitored at a peripheral health center until 25 weeks of gestation when the patient experienced a vaso-occlusive crisis, prompting her evacuation to the CHUR-OHG. Electrophoresis of hemoglobin revealed 53% hemoglobin S fraction and 47% hemoglobin C fraction, with mild anemia at 9.6g/dl. After symptomatic treatment and hydration, the first crisis improved; however, due to the succession of crises, manual exchange transfusion was discussed and indicated by the care team. Initially, this exchange was not performed due to a lack of blood products. Thanks to the opening of the new transfusion center in the region, the required blood bags were available for the procedure. Manual exchange transfusion was performed, using AA red blood cell concentrate. The immediate aftermath of the exchange transfusion was straightforward, and the patient was able to carry her pregnancy to term without further complications. Delivery was by caesarean section, at 38 weeks of gestation. The newborn, a female, had Apgar scores of 9/10 and 10/10, with a weight of 2600 grams. Postoperative recovery was uneventful with no vaso-occlusive crises.

Microemboli on Transcranial Doppler in Patients with Sickle Cell Disease: A Single Center Case Series

Background: Stroke remains a major cause of morbidity in patients with sickle cell disease (SCD). Current guidelines recommend that patients with Hemoglobin SS and Hemoglobin S beta zero thalassemia have annual transcranial doppler (TCD) screening and that patients with abnormal velocities should have regular blood transfusions to maintain a maximum HbS level <30% and maintain a hemoglobin level >9 g/dL for at least one year to decrease risk of stroke (DeBaun et al., 2020). While research has shown that patients with velocities >200cm/sec are at increased risk of cerebral infarct, there is no literature to discuss the follow up and management of patients with microemboli on TCD. In the past microemoli were unable to be detected, however TCD is now able to detect such findings (Wan et al., 2022). Studies have been completed on patients with ischemic stroke without SCD and have shown that the presence of microemboli on TCD is associated with a higher rate of recurrence (Das et al., 2020). The presence of emboli has also been shown to recognize patients with asymptomatic carotid stenosis and increased risk for stroke (Markus et al., 2010). Although noted in the general population, the significance of such a finding on an annual TCD screening in patients with sickle cell disease is unknown. Methods:At the Medical University of South Carolina, we performed a retrospective review of all patients with SCD who have had TCDs from 2017 to February 2023 and were found to have microemboli to evaluate management and outcome. Results: We have had 5 patients with an incidental finding of microemboli on their annual TCD since 2017. Three were male with median age of 3 (range 2 to 15 years). Four individuals are HbSS genotype. Mean hemoglobin at the time of TCD was 8.26 ± 1.4 g/dL. TCD velocity was normal in four patients at the time of the TCD, while the remaining individual had a low conditional TCD. Two have a previous history of conditional or abnormal TCDs. The location of the microemboli was variable among patients (Table 1). At the time of microemboli findings on TCD, discussions were held with family regarding options for treatment including chronic partial exchange transfusions and optimization of other disease modifying therapies. Management of all patients included MRI at the time of microemboli, which was normal in only two patients. Three patients were started on chronic manual exchange transfusions for 10 months to 3 years. One out of the 3 continued home HU while receiving transfusions while the remainder were transitioned to HU once chronic transfusions were complete. One patient was not started on chronic transfusions but monitored closely with TCDs due to family choice. On follow up, 4 have had repeat TCDs and all have normalized without any subsequent episodes of microemboli. One patient was found to have silent infarct on MRI at time of TCD and 1 has had follow up MRI 4 years after original TCD with a new silent infarct. Of note, this patient had inconsistent follow up with chronic transfusions after initial abnormal TCD. Importantly, none of our 5 patients had an acute symptomatic ischemic event since the microemboli were noted. Overall, among these patients, 2 have had concern for silent infarct and 3 of 5 started on chronic transfusions (Table 1). Conclusion: This is the first report within the literature of microemboli on annual TCD screening in patients with SCD. As this is a new phenomenon, the significance within the disease course is not known for those with sickle cell disease. Identifying patients with microemboli has been used to help navigate the need for early intervention to prevent stroke. Given that individuals with SCD are already at an increased risk for cerebrovascular events, these findings of microemboli could indicate further risk in these individuals as well and deserve further investigation. Further research is warranted as the significance of such a finding is unknown and further understanding could impact the standard of care of this patient population.

Plasmodium knowlesi (Pk) Malaria: A Review & Proposal of Therapeutically Rational Exchange (T-REX) of Pk-Resistant Red Blood Cells.

Plasmodium knowlesi (Pk) causes zoonotic malaria and is known as the "fifth human malaria parasite". Pk malaria is an emerging threat because infections are increasing and can be fatal. While most infections are in Southeast Asia (SEA), especially Malaysia, travelers frequently visit this region and can present with Pk malaria around the world. So, clinicians need to know (1) patients who present with fever after recent travel to SEA might be infected with Pk and (2) Pk is often misdiagnosed as P. malariae (which typically causes less severe malaria). Here we review the history, pathophysiology, clinical features, diagnosis, and treatment of Pk malaria. Severe disease is most common in adults. Signs and symptoms can include fever, abdominal pain, jaundice, acute kidney injury, acute respiratory distress syndrome, hyponatremia, hyperparasitemia, and thrombocytopenia. Dengue is one of the diseases to be considered in the differential. Regarding pathophysiologic mechanisms, when Pk parasites invade mature red blood cells (RBCs, i.e., normocytes) and reticulocytes, changes in the red blood cell (RBC) surface can result in life-threatening cytoadherence, sequestration, and reduced RBC deformability. Since molecular mechanisms involving the erythrocytic stage are responsible for onset of severe disease and lethal outcomes, it is biologically plausible that manual exchange transfusion (ET) or automated RBC exchange (RBCX) could be highly beneficial by replacing "sticky" parasitized RBCs with uninfected, deformable, healthy donor RBCs. Here we suggest use of special Pk-resistant donor RBCs to optimize adjunctive manual ET/RBCX for malaria. "Therapeutically-rational exchange transfusion" (T-REX) is proposed in which Pk-resistant RBCs are transfused (instead of disease-promoting RBCs). Because expression of the Duffy antigen on the surface of human RBCs is essential for parasite invasion, T-REX of Duffy-negative RBCs-also known as Fy(a-b-) RBCs-could replace the majority of the patient's circulating normocytes with Pk invasion-resistant RBCs (in a single procedure lasting about 2 h). When sequestered or non-sequestered iRBCs rupture-in a 24 h Pk asexual life cycle-the released merozoites cannot invade Fy(a-b-) RBCs. When Fy(a-b-) RBC units are scarce (e.g., in Malaysia), clinicians can consider the risks and benefits of transfusing plausibly Pk-resistant RBCs, such as glucose-6-phosphate dehydrogenase deficient (G6PDd) RBCs and Southeast Asian ovalocytes (SAO). Patients typically require a very short recovery time (<1 h) after the procedure. Fy(a-b-) RBCs should have a normal lifespan, while SAO and G6PDd RBCs may have mildly reduced half-lives. Because SAO and G6PDd RBCs come from screened blood donors who are healthy and not anemic, these RBCs have a low-risk for hemolysis and do not need to be removed after the patient recovers from malaria. T-REX could be especially useful if (1) antimalarial medications are not readily available, (2) patients are likely to progress to severe disease, or (3) drug-resistant strains emerge. In conclusion, T-REX is a proposed optimization of manual ET/RBCX that has not yet been utilized but can be considered by physicians to treat Pk malaria patients.

5567917 COST COMPARISON OF TRANSFUSION CHOICES IN OMAN FOR PATIENTS SUFFERING FROM SICKLE CELL DISEASE

Background: Sickle cell disease(SCD) management can be challenging, requiring different therapeutic approaches because SCD could induce multiple complications. Red blood cell(RBC) transfusion is well known as one of the disease-modifying therapies available for long-term management. It’s supported by multiple randomized clinical trials for the prevention of disease complications such as stroke in adults and children with SCD.RBCs can be delivered via three methods: Simple transfusion(ST), manual exchange(ME) and automated red blood cell exchange(aRBCX). In ST, patients receive additional units of blood. The purpose is to raise the haemoglobin (Hb) to a steady state and maintain the oxygen-carrying capacity of blood. ME transfusion consists of removing the patient’s sickle-shaped RBCs and replacing them with healthy ones— lowering the concentration of sickled cells without increasing blood viscosity. This is performed using repeated alternating isovolumetric phlebotomy and blood transfusion. aRBCX involves removing sickled RBCs from the patient and rapid replacement with healthy RBCs while maintaining isovolemia. An advantage of aRBCX is the avoidance or minimization of costly iron chelation therapy to treat iron overload. Aim: SCD management is an important cost driver for health systems. The intent of this research was to compare the cost-effectiveness of different transfusion modalities. We wanted to compare the all-inclusive costs. and wanted to perform this evaluation in Oman, as the incidence of SCD ranges between 0.2% and 0.07%. This disease is considered one of Oman’s most common genetic blood disorders. Method: In-depth data about variables that drive costs have been collected to populate a de novo cost model. We collected data from the Royal Hospital and Sultan Qaboos University Hospital. Cost drivers that we took into consideration were disease incidence, cost of blood transfusion, medical equipment, disposables, cost of staff, complications, iron chelation therapy, hospitalization, and lives lost. The assessment took place in June 2020. The cost of the treatment intervention, cost of disease-related complications and the yearly cost of treatment imputable to each transfusion modality represented the most significant differences across the three transfusion choices. By populating the model with resources provided by the two above-mentioned hospitals, we could demonstrate the cost differences between transfusion choices and the potential benefits that healthcare providers can experience. The results demonstrate significant differences across cost parameters, such as Cost of treatment, Cost of treatment-related potential effect, Cost of complications, Cost of life years lost, and Estimated total yearly cost per patient. Results and conclusion: aRBCX promotes a reduction in overall resource utilization and improved patient outcomes. aRBCX is approximately 47% less costly than ST, and is approximately 23% less costly when compared to ME. By highlighting the consequence of treatment choice and its financial impact, we aim to support the adoption of aRBCX in comparison to alternative transfusion choices and validate future reimbursement considerations. aRBCX cost savings may lead to better adoption. We recommend researchers rerun these calculations with the model on an individual hospital basis.

Comparative study between chronic automated red blood cell exchange and manual exchange transfusion in patients with sickle cell disease: A single center experience from Saudi Arabia.

Red cell transfusion remains the gold standard in managing sickle cell disease (SCD) with severe complications. Offering red blood cell exchange (RBCX) either manual exchange transfusion (MET) or automated RBCX (aRBCX) can reduce the complications of chronic transfusion and maintain target Hb thresholds. This study audits the hospital experience of overseeing adult SCD patients treated with RBCX, both automated and manual, and compares the safety and efficacy. This retrospective observational study was conducted as an audit for chronic RBCX for adult patients with SCD in 2015-2019 at King Saud University Medical City, Riyadh, Saudi Arabia. A total of 344 RBCX for 20 adult SCD patients who were enrolled in regular RBCX, (11/20) patients had regular aRBCX with a total of (157) sessions, and (9/20) patients had MET with a total of (187) sessions. The median level of HbS% post-aRBCX was significantly lower than MET (24.5.9% vs. 47.3%, P < 0.010). Patients on aRBCX had fewer sessions (5 vs. 7.5, P < 0.067) with better disease control. Although the median yearly pRBC units per patient for aRBCX was more than the double needed for MET (28.64 vs. 13.39, P < 0.010), the median ferritin level was 42 μg/L in aRBCX versus 983.7 μg/L in MET, P < 0.012. Compared to MET, aRBCX was more effective in reducing HbS, with fewer hospital visits and better disease control. Although more pRBCs were transfused, the ferritin level was better controlled in the aRBCX group without increasing alloimmunization risk.

P-072: COST COMPARISON OF TRANSFUSION CHOICES IN OMAN FOR PATIENTS SUFFERING FROM SICKLE CELL DISEASE.

Purpose: The management of sickle cell disease (SCD) can be challenging, requiring different therapeutic approaches because SCD could induce multiple complications, precipitating multi-organ failure. Red blood cell (RBC) transfusion is well known as one of the disease-modifying therapies available for long-term management of this condition. It is supported by multiple randomised clinical trials for the prevention of disease complications such as stroke in adults and children with SCD. RBCs can be delivered via three methods: Simple transfusion(ST), manual exchange(ME) and automated red blood cell exchange(aRBCX). In a simple transfusion, patients receive additional units of blood. The purpose is to raise the haemoglobin (Hb) to a steady state and maintain the oxygen-carrying capacity of the blood. Manual exchange transfusion removes the patient’s sickle-shaped RBCs and replaces them with healthy ones — lowering the concentration of sickled cells without increasing blood viscosity. This is performed using repeated alternating isovolumetric phlebotomy and blood transfusion. Automated red blood cell exchange transfusion involves removing sickled RBCs from the patient and rapid replacement with healthy RBCs while maintaining isovolemia. An advantage of aRBCX is avoiding or minimising costly iron chelation therapy to treat iron overload. As Sickle Cell disease (SCD) management is a critical cost driver to health systems, this research intended to compare the cost-effectiveness of different transfusion modalities. We wanted to compare the following transfusion types: Automated red blood cell exchange (aRBCX), Top-up transfusion and Manual red blood cell exchange (mRBCX). More specifically, we wanted to perform this evaluation in Oman as 0.2% of the Omani population suffers from this disease, which is the most frequent genetic disorder with the highest incidence. Materials and methods: Variables that drive costs have been collected to populate a de Novo cost model. We collected data from the Royal Hospital and Sultan Qaboos University Hospital. The most significant cost drivers which we took into consideration were: disease incidence, cost blood transfusion, medical equipment, disposables, complications, chelation therapy, hospitalisation, lives lost. Results: Our results reflect a 5-year overview demonstrating the costs and benefits experienced by healthcare providers. When considering the cost of treatment, consequence, complications, lives lost, life years lost, aRBCX is dominant over the transfusion modalities. Yearly intervention cost per patient per year in Omani-Rial: [Top-up 1933.5;Manual 3101.5;aRBCX 5857.5].The yearly cost of treatment choice related cost per patient per year in Omani-Rial:[12128.88; 6233.41; 789.88].Cost of disease complications per patient per year in Omani-Rial:[12706;12864; 11346].Cost of lives lost per patient per year in Omani-Rial:[1315;1288;1102). The total yearly cost per patient per year in Omani-Rial is estimated:[28084;23487.5;19095] Conclusion: aRBCX is 47% more cost-effective when compared to Top-up transfusion. aRBCX is 23% more cost-effective when compared to mRBCX. aRBCX promotes reduction in overall resources, disease burden, bed retention and the need for chelation therapy when compared to top-up transfusion and manual exchange transfusion. Cost savings may lead to better adoption. We recommend researchers rerun these calculations on an individual hospital basis.The authors do not declare any conflict of interest

Comparative evaluation of efficacy and safety of automated versus manual red cell exchange in sickle cell disease: A systematic review and meta-analysis.

Exchange transfusion is a valuable treatment option in sickle cell disease (SCD) and is preferred over simple transfusion as it removes abnormal haemoglobin S (HbS) levels and reduces complications. This meta-analysis aims to evaluate the efficacy and safety profile of automated red cell exchange (aRBX) procedure over manual red cell exchange transfusion (MET) in SCD patients. A standard meta-analysis protocol was developed, and after performing a comprehensive literature search in PubMed/MEDLINE, Cochrane and International Clinical Trial Registry Platform (ICTRP), reviewers assessed eligibility and extracted data from nine relevant studies. A random effects model was used to estimate the pooled effect size calculated from the mean difference in HbS percentage, serum ferritin level and risk ratio for the adverse events. Quality assessment was done using the risk-of-bias assessment tool, and a summary of observations was prepared using standard Cochrane methodology with GradePro GDT. The random-model analysis revealed a mean difference of 4.10 (95% CI: -3.29-11.49;Z= 1.09;p= 0.28) for HbS percentage, mean difference of 435.29 (95% CI: -73.74-944.32;Z= 1.68;p= 0.09) for serum ferritin and pooled risk ratio of 1.35 (95% CI: 0.63-2.87; Z=0.77;p= 0.44) for adverse events. This meta-analysis did not reveal any significant benefit of aRBX in reducing HbS percentage and attenuating the serum ferritin level when compared with MET. There was also no significant increased risk of adverse events detected in association with aRBX.

I Got It from My Mamma: Sensitization by Transplacental Transfer of Maternal HLA Antibodies Managed with Manual Exchange Transfusion

<h3>Introduction</h3> During pregnancy antibodies are passed from mother to fetus with many known immunologic benefits. There is no data about the implications of this on pediatric transplant immunology. We report a case of transplacental transfer of maternal Human Leukocyte Antigen (HLA) antibodies affecting cardiac transplant candidacy in an infant with cardiomyopathy. <h3>Case Report</h3> A 3-week-old Amish boy with a homozygous MYBPC3 mutation causing severe cardiomyopathy admitted for refractory heart failure symptoms was referred for heart transplant evaluation. Labs were notable for high levels of high Mean Fluorescence Intensity (MFI) HLA antibodies (Panel of Reactive Antibodies (PRA) Class I: 74%, Class II: 80%) with no known sensitizing events. Maternal history was remarkable for multiple miscarriages raising the question that his HLA antibodies could be a result of maternal HLA sensitization with transplacental antibody transfer versus active antibody production by the child. Subsequent antibody testing of his mother's blood and breast milk revealed nearly identical HLA antibody profiles to that of the patient. Given the patient's prohibitively small size for antibody removal by plasmapheresis, we elected to perform double volume whole blood exchange transfusions with serial monitoring of his HLA antibodies. Considering his severe ventricular dysfunction, exchange transfusions were performed manually via the push pull method over 6 hours. He ultimately underwent 6 exchange transfusions over the course of 7 weeks. Serial antibody testing revealed a progressive decline in all his HLA antibody levels to <3,000 MFI with only mild rebound after each transfusion consistent with tissue redistribution rather than active antibody production. He was successfully transplanted with a negative PRA (Class I: 0%, Class II: 0%) without post-transplant complications. <h3>Summary</h3> We believe this is the first report demonstrating transplacental acquisition of maternal HLA antibodies in an infant successfully removed via serial whole blood exchange transfusions enabling successful cardiac transplantation.

Adding Hydroxyurea to Chronic Transfusion for Sickle Cell Anemia Reduces Transfusion Burden: Final Results of the HAT Prospective Trial

Background: Chronic red blood cell (RBC) transfusion is an established therapy to prevent stroke in patients with sickle cell anemia (SCA). Chronic transfusion, however, requires considerable blood resources and has risks so a strategy to decrease transfusion requirements is highly desirable especially given blood shortages. Chronic transfusion also can fail to prevent progressive cerebrovascular disease so further optimization of this therapy is needed. Hydroxyurea has been studied as an alternative to chronic transfusion, but alone it provides inadequate cerebrovascular protection for patients at highest risk for stroke. Combination therapy hydroxyurea and transfusion (HAT) may provide benefits over either therapy alone because each ameliorates the pathology of SCA via different mechanisms. To evaluate the feasibility and impact of HAT on transfusion requirements, we conducted a single-arm clinical trial (NCT03644953).Methods: Pediatric patients with SCA already receiving simple chronic transfusion for stroke prevention for at least one year were approached. Patients with poor adherence to chronic transfusion defined by having a hemoglobin (Hb) S >45% and a transfusion interval >5 weeks in the last year were excluded. Enrolled patients were started on hydroxyurea 20 mg/kg/day and continued on simple chronic transfusions with the same transfusion protocol. Transfusion volumes were based on the pre-transfusion Hb to achieve a target post-transfusion Hb of 11.5-12.0 g/dL. Hydroxyurea was dose escalated to achieve a HAT target dose (HAT-TD) defined by either an absolute neutrophil count <4000/µL, or a pre-transfusion Hb >10g/dL with HbS >30%. Transfusion volumes and pre-transfusion laboratory values were compared the year before HAT and the year after HAT-TD.Results: A total of 19 eligible patients were offered participation and 14 enrolled (74% recruitment ratio). The median age was 11.2 years (range 5.3-19.8). The primary indication for chronic transfusion was history of an abnormal transcranial Doppler ultrasound (n=7), overt stroke (n=6), and progression of cerebral vasculopathy (n=1). Five participants ended the study prematurely (care transferred to another hospital, nonadherence to transfusions, patient desire to not take medications, hair thinning, family concern after a hospitalization); none had a serious adverse event that was attributed to HAT. Among the 9 participants who completed the study, the median HAT-TD was 25 mg/kg/dose (range 20-30) and was achieved after a median of 18 weeks (range 0-26). The median hydroxyurea adherence ratio, measured by pill counts, was 91%. One patient with baseline splenomegaly developed splenic sequestration requiring hospitalization; there were no other serious adverse events attributed to HAT. One patient developed a pre-transfusion Hb >11 g/dL and HbS >45%. This patient had hydroxyurea held for 12 weeks and was changed to partial manual exchange transfusions. With HAT, patients had a significant increase in pre-transfusion total Hb, HbS, HbF, and MCV; and a significant decrease in ANC and LDH (Table). After HAT-TD, 8/9 participants received fewer RBCs with a significant median RBC volume reduction of 19.4 ml/kg/year (p=.02, Figure). The single patient who received more RBCs after HAT-TD had suspected nonadherence to hydroxyurea returning only 33% of dispensed hydroxyurea bottles. While receiving HAT 8/9 patients had brain magnetic resonance imaging for clinical monitoring after a median of 75 weeks (range 29-100), no patient had a new infarct or progressive vasculopathy.Conclusion: Hydroxyurea added to chronic transfusion therapy for patients with SCA is feasible. HAT effectively decreases transfusion volumes so can help conserve blood resources. While Hb S% increases with HAT, this increase is relatively small and markers of hemolysis improved. Most patients are able to safely continue simple transfusions when hydroxyurea is added, but a few may require exchange transfusions. The effect of HAT on cerebrovascular disease warrants further study. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Manual Partial Exchange Transfusion a Cost Effective Life Saving Intervention in Sickle Cell Crisis: Single Centre Retrospective Data Analysis

Introduction: Sickle cell disease (SCD) poses a considerable health burden in lower middle income country (LMIC) like India. One ICMR survey reported about 20% of children with SCD died by the age of two years and 30% of children with SCD amongst the tribal community in India died before they reached adulthood. Data on mortality rate in the adult population with SCD is sparse. Our centre data shows that despite the availability of Hydroxyurea and supportive care, almost one-third of hospitalized Sickle Cell Crisis patients develop life threatening complications. Manual Partial Exchange Transfusion is a cost-effective intervention to decrease mortality in Sickle Cell Crisis. But perhaps, it is an underutilized therapy. In India, the cost incurred for each session of exchange transfusion, if done by the RBC apheresis machine, is $420. Whereas Manual Exchange Transfusion costs only $70(including the cost of the central venous catheter) for the first session followed by $15 for each subsequent session. Aim: A single centre retrospective data analysis to evaluate the outcome of Sickle Cell Crisis patients undergoing Manual Partial Exchange Transfusion during hospitalization. Material and Method: A total of 553 SCD patients on regular follow up at the Haemato Oncology Care Centre (HOCC) from July 2012 to July 2020 were evaluated.169 patients were hospitalized for treatment of Sickle Cell Crisis at the Bhailal Amin General Hospital and Sterling Hospital Vadodara. The data was retrieved after the IRB/Apex committee approvals for retrospective analysis. The indication for exchange transfusion was Acute Chest Syndrome 19(35%), Hepatic cell crisis 11(20%), Vaso Occlusive Crisis not responding to the conservative line of therapy in 48 hours of treatment 11(20%), Cerebrovascular Accident (CVA) 5 (9%), Avascular Necrosis of Femur with excruciating pain 3 (6%), Complicated Dengue with Multi-organ failure 3 (6 %), Priapism 1 (2%) and Splenic Sequestration 1 (2%). Central venous access was secured with a central venous catheter or dialysis catheter. Simple packed cell volume was transfused to 11(20%) patients and 5(9%) patients received platelet transfusion. The cut-off values for HB and Platelet count were 8 gm% and 50000/cumm, respectively at the time of the Manual Partial Exchange Transfusion procedure. Blood volume withdrawn was 5 to 10 ml /kg, followed by an equal volume of packed cell volume transfusion at every session. The procedure was repeated every 12 hr or 24 hr depending on the clinical condition of the patient. HbS value was reassessed post 4 sessions with repeat testing done after 2 to 4 sessions if the observed HbS value was more than 30% or more than 10% (for CVA) after 4 sessions. The endpoint was clinical recovery with HbS less than 30% or no clinical recovery despite the achievement of HbS less than 10%. In CVA the endpoint was HBS less than 10%. Results: Manual Partial Exchange Transfusion was carried out in 54/169 (32 %) hospitalized patients. The median age was 25yrs (range 5 yrs to 69 yrs), with male predominance [Males 42(77%) and females 12(23%)].Pre procedure HbF value was &amp;lt;10% in 13(24%), 10 to 20% in 23 (43%) and &amp;gt;20% in 18(33%). A total of 50 out of 54 (93%) patients recovered completely. 28 (52%) patients were hemodynamically stable with normal SPO2 on room air at the time of Manual Partial Exchange Transfusion with an average of 7 days of hospitalization. All of these patients recovered completely with less than 5 sessions of Manual Partial Exchange Transfusion. 26(49%) patients were critically ill and had an average of 12 days of hospitalization. They were on Ventilator and Inotrope support at the time of Manual Partial Exchange Transfusion. 14/26(54%) critically ill patients recovered completely with an average of 6 sessions of Manual Partial Exchange Transfusion. 12/26(46%) critically ill patients succumbed even though post exchange HBS value decreased to less than 10%. The overall mortality rate of SCD patients in this analysis was 12/553 (2.1%), significantly lower than what was historically reported as 30% in the ICMR survey. Underlying Dengue viral infection associated with Multi-organ dysfunction and Fulminant hepatic failure were risk factors for mortality observed in our study. Conclusion: Manual Partial Exchange Transfusion is highly effective in reducing mortality in Sickle Cell Crisis. It is feasible and cost-effective in small centres lacking apheresis machine facility. Disclosures No relevant conflicts of interest to declare.

Automated exchange compared to manual and simple blood transfusion attenuates rise in ferritin level after 1 year of regular blood transfusion therapy in chronically transfused children with sickle cell disease

Optimal strategies for regular blood transfusion therapy are not well defined in sickle cell disease (SCD). This analysis tested the hypothesis that in the first of year of regular transfusions, when chelation therapy use is minimal, automated exchange transfusion would be the superior method for attenuating the rise in ferritin levels compared to simple and manual exchange transfusions. The Silent Cerebral Infarct Multi-Center Clinical Trial randomly allocated children with SCD and silent cerebral infarcts to receive standard care or regular transfusions with a target pre-transfusion HbS concentration ≤ 30% and minimum hemoglobin level > 9.0 g/dL. Participants with at least nine transfusions and sufficient ferritin testing in the first year of the trial were included in a planned secondary analysis. Ferritin levels by the end of the first study year were compared between participants receiving automatic exchange transfusion, manual exchange transfusion, and simple transfusion. A total of 83 participants were analyzed. During the first year of the study, 75.9% of the participants had >80% of transfusions via one transfusion method. At baseline no significant differences in ferritin levels were observed in the three transfusion groups (p = 0.1). After 1 year of transfusions the median (interquartile range) ferritin levels in the simple transfusion (n = 40), manual exchange transfusion (n = 34) and automatic exchange transfusion (n = 9) groups were 1800 ng/mL (1426-2204 ng/mL), 1530 ng/mL (1205-1805 ng/mL), and 355 ng/mL (179-579 ng/mL), respectively (p < 0.001). Automated exchange transfusion, when compared to other transfusion methods, is the optimal transfusion strategy for attenuating increase in ferritin levels in children with SCD.

Rate of Sickle Hemoglobin Recovery in Sickle Cell Disease Patients Undergoing Red Blood Cell (RBC) Exchange Transfusion Is Associated with Age of Patients and Number of RBC Units Transfused

Rate of Sickle Hemoglobin Recovery in Sickle Cell Disease Patients Undergoing Red Blood Cell (RBC) Exchange Transfusion is Associated with Age of Patients and Number of RBC Units Transfused Introduction: Automated and manual red blood cell exchange (RBCX) transfusions are useful in the primary and secondary prevention of sickle cell disease (SCD) complications (Ware et al., 2012). The ability to consistently maintain sickle hemoglobin (HbS) below target (30% or 50% depending on indication) is quite variable (Kuo et al., 2012). With emerging indications such as silent cerebral infarction, it is imperative that effective means of chronic transfusion to maintain appropriate hematological and clinical targets be identified. We hypothesize the rate of HbS recovery is dependent on the individual's hemolytic and erythropoietic rate. The purpose of this study is to evaluate the effect of the rate of erythropoiesis and hemolysis on HbS recovery in SCD patients undergoing RBCX. Methods: Fifteen (15) patients were prospectively recruited from the adult SCD transfusion program (9 automated, 6 partial manual), from December 2018 to July 2019, and followed through one exchange cycle (4 weeks). Automated and partial manual exchange transfusion protocols have been previously described elsewhere (Canadian Haemoglobinopathy Association Consensus Statement on the Care of Patients with Sickle Cell Disease in Canada, Version 2.0, Ottawa; 2015). Exclusion criteria included active hydroxyurea or erythropoietic stimulating agents use, reported ill health in the preceding 4 weeks, co-morbid hemolytic condition or non-HbSS genotype. Hemoglobin, hematocrit, HbS, lactate dehydrogenase (LDH), reticulocyte count, indirect bilirubin, and serum erythropoietin level were determined for each patient: pre- and post- first exchange, weekly for 3 weeks and pre- second exchange (the 4th week). Descriptive variables were either expressed as means ± SD or median (IQR), based on normality, while linear regression was performed for continuous variables. Co-variates were included in multivariable analysis if P &lt; 0.10. Multivariable linear regression was conducted to examine the potential association between the change in HbS over one RBCX cycle and age of patients, pre-RBCX hematocrit, LDH, and number of RBC units transfused. Results: We identified 36 eligible patients from the Program database, after which 15 consented to participate in the study. Mean age was 32.9 ± 12.3 years, consisting of 7 males and 8 females. There was an association between the rate of change in HbS and age of patients (p=0.035), pre-RBCX hematocrit (p=0.030) and number of transfused RBC units (p=0.030). LDH showed a trend towards reduced rate of change in HbS (p=0.069). Rate of change in HbS was not associated with automated vs. partial RBCX (Figure), female vs. male patients, pre-RBCX HbS, erythropoietin, indirect bilirubin, reticulocyte and age of transfused RBCs. Age of patients (p&lt;0.001) and number of units transfused (p=0.010) were independently associated with the rate of change of HbS, after adjusting for hematocrit and LDH. For every decade increase in age, the rate of HbS recovery was 3% lower in one RBCX cycle. For each additional unit of RBC exchanged, the rate of HbS recovery was 0.78% higher in one RBCX cycle. Conclusion: This is the first study to evaluate the determinants of variability in the rate of HbS recovery in SCD patients on RBCX. Age of patients and number of RBC units transfused may underlie the significant variability in achieving HbS targets in SCD patients on RBCX. We failed to show a significant difference in the rate of change in HbS between automated and partial manual exchange transfusion. Reduction in HbS recovery with advancing age may be related to erythropoietic reserve in the patients' marrow. However, the influence of units of RBC recovery appeared paradoxical, and the finding may be spurious due to small sample size. It may therefore be possible to appropriately titrate the number of RBC units with advancing age of SCD patients with a view to achieving desired HbS targets. Disclosures Patriquin: Ra Pharma: Consultancy, Research Funding; Apellis: Consultancy, Honoraria, Research Funding; Alexion: Consultancy, Honoraria, Research Funding; Octapharma: Consultancy, Honoraria, Research Funding. Kuo:Agios: Consultancy; Alexion: Consultancy, Honoraria; Apellis: Consultancy; Bioverativ: Other: Data Safety Monitoring Board; Bluebird Bio: Consultancy; Celgene: Consultancy; Novartis: Consultancy, Honoraria; Pfizer: Consultancy.

Identification of the Pitfalls in the Attempted Use of Extracorporeal Membrane Oxygenation in an Adult Patient with Sickle Cell Disease and Severe Acute Chest Syndrome

BACKGROUNDAcute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). Despite being the leading cause of death in adult patients with SCD, current recommendations for treatment of ACS remain largely supportive, consisting of pain management, aggressive fluid resuscitation, respiratory support, and transfusion therapy. Despite these measures, it is not uncommon for patients to require intubation due to progression to acute respiratory distress syndrome (ARDS). Recently, there have been a number of case reports that have successfully utilized extracorporeal membrane oxygenation (ECMO) for the management of ACS in those patients who fail to respond to conventional therapy [Kuo et al., 2013, Sewaralthahab et al, 2018]. However, the use of ECMO in this patient population remains uncommon, and further evaluation of this intervention is needed. This case report details an unsuccessful attempt at the use of ECMO in the case of ARDS secondary to ACS, in an attempt to identify critical pitfalls.CASE REPORTA 32-year-old African-American female with HbSS disease on hydroxyurea therapy was transferred from an outside hospital following 3 days of respiratory decompensation. Prior to arrival, patient coded once at the outside hospital and once on transfer. Veno-arterial ECMO was initiated with improving oxygen saturation and volume status with continuous renal replacement therapy. To maintain an ECMO-specific goal hemoglobin level of 10 g/dL, 1:1 manual exchange transfusions were performed due to an inability to access equipment for automated RBC exchange. Once stable enough for CT, patient was found to have gray-white inversion suggestive of irreversible severe brain damage. Following another 28 days of supportive care with no neurologic improvement, the family decided to withdraw care, and the patient expired.CONCLUSIONWhile unsuccessful, this patient's case revealed a need for defining parameters regarding the initiation of ECMO in SCD patients with severe ACS. A review of previously-published literature has shown that the use of ECMO for the management of ARDS in adults is more efficacious than conventional ventilation support [Peek et al., 2009]. In patients with SCD, this improvement in efficacy is not readily reproduced, likely due to unique challenges presented by the pathophysiology of the disease. Notably, patients with SCD face additional risks of venous thromboembolism and strokes while on prolonged bed rest due to a baseline prothrombotic state [Sewaralthahab et al., 2018]. A systematic review of available case reports is needed to develop a protocol for the management of severe ACS that takes SCD-specific risks into account.The present report also makes a case for the training of providers in the early recognition of severe ACS in SCD patients. SCD remains largely undertreated in the United States, likely due to a complex interplay of patient, physician, and institutional factors. Had this patient been transferred immediately to a facility better equipped to provide a higher level of care, her condition could have arguably taken a different course. Despite the aforementioned challenges, ECMO remains a feasible option for the management of severe ACS in patients with SCD, and efforts should be made to standardize current treatment protocols.REFERENCESKuo KW, Cornell TT, Shanley TP, Odetola FO, and Annich GM. The use of extracorporeal membrane oxygenation in pediatric patients with sickle cell disease. Perfusion. 2013 September; 28(5): 424-432.Peek GJ, Mugford M, Tiruvoipati R, Wilson A, Allen E, Thalanany M, et al. Efficacy and economic assessment of conventional ventilatory support versus extracorporeal membrane oxygenation for severe adult respiratory failure (CESAR): a multicentre randomised controlled trial. The Lancet. 2009 October; 374(9698): 1351-1363.Sewaralthahab SS, Menaker J, Law JY. Successful use of veno-venous extracorporeal membrane oxygenation in an adult patient with sickle cell anemia and severe acute chest syndrome. Hemoglobin. 2018 42(1): 65-67.DisclosuresNo relevant conflicts of interest to declare.

PB2311 COMPARISON BETWEEN THE EFFECTIVENESS OF CHRONIC PARTIAL RED BLOOD CELL EXCHANGE AND AUTOMATED RED BLOOD CELL EXCHANGE TRANSFUSION IN SICKLE CELL DISEASE: THE EXPERIENCE OF A HOSPITAL IN PORTUGAL

Background:In sickle cell disease (SCD), red blood cell (RBC) transfusion has an important role in the management of emergency and elective settings. In both settings, transfusion may be administered by simple transfusion or by exchange transfusion, manual exchange transfusion or automated Exchange transfusion (AET). Chronic Transfusion Program (CTP) remains the gold standard therapy for stroke prevention and for patients with a severe disease who have inadequate response to hydroxyurea treatment.Aims:To evaluate the safety, efficacy and cost between the patients that underwent both AET and partial manual exchange transfusion (pMET) procedures.Methods:Retrospective observational cohort study of patients with SCD on CTP that have switched between pMET and AET. This study was carried out from 01/01/2017 to 31/12/2018 in a Tertiary Hospital in Portugal. Data on patient history, biological values, duration of the procedure, intervals between them, adverse events as well as the cost of material and human needs were collected and compared between both proceduresResults:A total of 6 patients met the inclusion criteria described. However, 1 patient was excluded from our study because of the lack of attendance to the CTP. During the study, we recorded 88 exchange procedures (42 pMET and 46 AET), both on peripherical venous access. From all those procedures the major concern was the poor venous access, which was the reason why 2 patients had returned to pMET. No major complication or alloimmunization was observed. The indications for CTP were cerebral vasculopathy (N = 2), stroke (N = 1) and recurrent vaso‐occlusive crisis with multiorgan failure (N = 2). For both procedures, target values were to obtain a pre‐exchange HbS level ≤30% for stroke and cerebral vasculopathy and ≤50‐60% for other indications. We documented in 11,4% of patients a higher HbS level prior to the next procedure (N = 10). Despite that all patients remained stable without any SCD related event. Both procedures were well tolerated and iron overload was well controlled. The duration of the exchange procedure was longer and the intervals between procedures were shorter with pMET. Annual RBC requirements per procedure were superior and the overall costs related with AET were 2,2 times higher – 18.180,93€ and 8.174,79€ AET and pMET, respectively (estimated cost per session AET: 790,48€ and pMET:389,28€). Since in our cohort we did not observe the interruption of chelation due to the switch, we did not include the cost for chelation.Summary/Conclusion:Our study shows, as described in literature, that AET allows a faster decrease in the HbS level immediately after exchange transfusion than pMET. Nonetheless, the HbS level before both procedures, performed during the same interval, were similar. We verified that pMET has a comparable efficacy with AET in terms of preventing the development or progression of chronic complications and that the cost per procedure is significantly higher with AET. However, in a clinical situation where it is important to rapidly reduce the HbS level, and/or where the control of the target HbS is stricter so that the patients are clinically controlled without an increase in hospital visits, AET is preferred. We conclude that AET is more effective in the rapid reduction of HbS and ferritin levels, as well as being less time consuming. Despite this, for the reasons described above, it is more cost‐effective to maintain both AET and pMET procedures.

Successful Treatment of Acute Chest Syndrome with Manual Exchange Transfusion in a Patient with Sickle Beta&lt;sup&gt;+&lt;/sup&gt;-thalassemia

Acute chest syndrome (ACS), characterized by fever, respiratory symptoms, and new pulmonary infiltration, is a serious complication of sickle cell disease (SCD). Regardless of the etiology, the conventional treatment options for ACS include empirical antibiotic therapy, the administration of analgesics, and red cell transfusion. The indications and methods of red cell transfusion are critical. We herein report the case of a 26-year-old African-American man with SCD who developed ACS and who was successfully treated with manual exchange transfusion. Despite increasing globalization, SCD remains extremely rare in Japan. Manual exchange transfusion can be performed easily anywhere and should be considered for treating SCD patients presenting with ACS.

Comparison between Automated Erythrocytopharesis (AECP) and Manual Exchange Transfusion (M-Ex)) in Reducing Hb-S and in Recovery of Acute Chest Syndrome and Other Acute Presentations of Sickle Cell Disease Patients

Background:Sickle Cell Disease represents a national health problem in Saudi Arabia with close to 150 thousands of the population are afflicted with the disease. Patients are frequently admitted with life threatening complications like Acute chest syndrome (ACS), Hyperhemolysis (HH), pneumonia, thromboembolism including pulmonary Artery Thrombosis (PAT) or severe protracted painful vasoocclusive crises (VOC). Rapid lowering of Hemoglobin S helps in reducing sickling and in alleviating such complications and allowing rapid recovery. Exchange transfusion is the fastest way to remove pathogenetic sickling red blood cells and reducing Hb S level to a safe level. It can be done manually (Mex) or via Automated Erythrocytopharesis machine (AECP). In this study we compared manual exchange to AECP in achieving the targeted lowering of Hb S and in accelerating clinical recovery.Patients and Methods:Patients included are sickle cell disease patients (HbSS, HbSC, Hb S/thal) admitted to the ER of a central Hospital. Indications of exchange were: acute chest syndrome, acute severe painful vaso-occlusive crises refractory to standard ER protocol of analgesia, stroke, priapism, Hyperhemolysis, and acute pulmonary embolism.P value of significance was calculated using student t-test comparing between median Hb S achieved after manual exchange vs AECP.To assess the rapidity of reversal of desaturation in acute chest syndrome patients, the cumulative incidence of reversal of desaturation and normalization of Oxygen saturation on room air were plotted against time at 0 time of the start of exchange, 2 hours,4 , 12, 24, 48 and 72 hours/discharge (D/C)Results:Table 1 shows clinic-biological characteristics of patients who underwent exchange transfusion. A total of 230 patients-admissions were registered between Dec 1. 2017 to July 27, 2018 for painful VOC to ER; 51 (32%) had clinical indications for exchange (ACS 25, Stroke / fits 1, priapism 1, pulmonary artery thrombosis 1, Hyperhemolysis with VOC (n:7) , VOC with HLH (n:1), and the remaining with “refractory” painful VOC with or without infection. One patient died immediately at the time of arrival to ER before starting any standard resuscitative measures .Exchange transfusion was indicated and done for 53 (23%); 12 (22,6%) AECP and the remaining (77.4%) had Manual Exchange.The median Hb S after manual exchange was 44 % (range 31-74%) which was unsatisfactory and way higher than the targeted level while Automated ECP reached down satisfactorily to a median of 31%(range 8%-50%) ; 67% of whom achieved it with only one session. No mortalities or major procedure related complication reported with manual or automated ECP. Procedurally, 3 patients needed 2 automated sessions and 1 patient used 2 kits for one session. Manual exchange could not achieve the target Hemoglobin S level below or around 30% due to logistic and technical difficulties and sometimes patients' refusal while Automated ECP reached to a mean Hb s level of 28%( range 8%-50%) and nearly two thirds (67%) reached to as low as 31% Hb S level with only one session of Automated ECP and was associated with rapid improvement of the oxygenation within the first 2 hours of the procedure.Conclusions:Erythrocytopharesis (Automated RBC exchange) is effective, quick and safe procedure that is life saving for many patients with ACS and is associated with less difficulties and complications if compared with the manual exchange. Because SCA is a national problem in Saudi Arabia and acute chest syndrome and other acute major complications comprehensively kill SCD patients, Automated Erythocytopharesis should be available nation-wide like dialysis machines at all large hospitals in all cities and should be distributed according to the prevalence of SCA in the area or location. DisclosuresNo relevant conflicts of interest to declare.

Optimal Hemoglobin Level for Pediatric Sickle Cell Patients Who Undergo Adenotonsillectomy

Background:The prevalence of obstructive sleep apnea syndrome (OSA) in children with sickle cell disease (SCD) is higher than in the general pediatric population. Adenotonsillectomy involves significantly increased risks in patients with SCD because of the need for general anesthesia,which could induce a vaso-occlusive event. Various preoperative regimens have been suggested to reduce peri-operative risks for SCD patients.Objective: We retrospectively reviewed the perioperative management for pediatric SCD patients undergoing adenotonsillectomy. We sought to identify the optimal preoperative hemoglobin (Hb) level for patients to have lowest risk of post-operative complications.Methods: Children between 1 and 18 years old with SCD including HbSS, HbSC, HbS beta thalassemia (Sβ0 and Sβ+) who underwent adenotonsillectomy from 2007 to 2017 were identified at Holtz Children's Hospital. Patients without these diseases who underwent the procedures were collected as a control. The study was approved by the University of Miami Institutional Review Board (IRB). De-identified data were exported to GraphPad Prism version 5.02 to perform the statistical analyses. Means were analyzed by student t test, rates by Chi-squared test. p < 0.05 is considered statistically significant.Results: Thirty-four patients with SCD (mean 7.1 yrs) and 145 controls (mean 7.0 yrs) were identified with adenotonsillectomy (see Table 1). SCD patients had significant longer hospital stay (2.6 versus 0.9 days, p< 0.001), and higher postoperative complication rates than the control group (20.6% vs 4.0%, p= 0.02). Most of the sickle cell patients' complications were hypoxemia (5 out of 7), except one supraventricular tachycardia and one headache. Four out of 7 patients with pretransfusion (pretrx) Hb level above 10 g/dL received manual exchange transfusion to keep Hb over 10 g/dL. None had complication or PICU stay. When patients' pretrx Hb levels was 9-10 g/dL, 5/8 received transfusion without significant complications, with the goal of not increasing post transfusion Hb over 11.5 g/dL. Most patients with pretrx Hb level 7-9 g/dL received simple blood transfusion. For patients with preoperative Hb level 9-11.5 g/dL, the PICU transfer rate was significantly lower than ones with Hb <9 or >= 11.5 g/dL (11.5% vs 60%, p=0.04; see Figure 1). Complication rates were also lower in the Hb 9-11.5 g/dL group, but without statistical difference (19.2% vs 40%, p=0.3). Data is limited by small sample size in the SCD group.Conclusion(s): We concluded that for pediatric SCD patients planning for adenotonsillectomy, it is better to keep the posttransfusion Hb level at least above 9 mg/dL but less than 11.5 g/dL before procedure to avoid significant post operational complications. If pretransfusion Hb level is above 9 mg/dL, exchange transfusion by partial phlebotomy or simple transfusion (not to exceed 11.5 g/dL) may be considered. DisclosuresNo relevant conflicts of interest to declare.

Manual RBC exchange transfusion in a patient of sickle-beta thalassemia syndrome presenting in crisis: a case report

Sickle cell disease and beta thalassemia are caused by abnormal haemoglobin (Hb) derived from mutation of the HBB gene encoding beta-globin. Compound heterozygous status for both mutations results in HbS/beta thalassemia (Sickle- beta thalassemia). Vaso-occlusive phenomena and haemolysis are the clinical hallmarks and major causes of mortality. Here we report a case of successful reduction of HbS level by manual RBC exchange transfusion. Capillary zone electrophoresis showed the case to be Sickle-beta thalassemia. A total of 3 units of 450ml whole blood units were used for manual exchange transfusion done in 2 sittings on consecutive days. Preexchange HbS level was 80.9% of total Hb. HbS level after 24 hours of the second procedure was 44%. In the absence of facility to conduct automated RBC exchange by a cell separator, to reduce HbS in patients presenting with acute complications of SCD and in patients with Vaso-occlusive Crisis, previous stroke, manual RBC exchange can provide a better relief.

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload.

Children with sickle cell anemia (SCA) may be at risk of cerebral vasculopathy and strokes, which can be prevented by chronic transfusion programs. Repeated transfusions of packed red blood cells (PRBCs) is currently the simplest and most used technique for chronic transfusion programs. However, iron overload is one of the major side effects of this therapy. More developed methods exist, notably the apheresis of RBC (erythrapheresis), which is currently the safest and most efficient method. However, it is costly, complicated, and cannot be implemented everywhere, nor is it suitable for all patients. Manual exchange transfusions combine one or more manual phlebotomies with a PRBC transfusion. At the Reference Center of Sickle Cell Disease, we set up a continuous method of manual exchange transfusion that is feasible for all hospital settings, demands no specific equipment, and is widely applicable. In terms of HbS decrease, stroke prevention, and iron overload prevention, this method showed comparable efficiency to erythrapheresis. In cases where erythrapheresis is not available, this method can be a good alternative for patients and care centers.

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Children with sickle cell anemia (SCA) may be at risk of cerebral vasculopathy and strokes, which can be prevented by chronic transfusion programs. Repeated transfusions of packed red blood cells (PRBCs) is currently the simplest and most used technique for chronic transfusion programs. However, iron overload is one of the major side effects of this therapy. More developed methods exist, notably the apheresis of RBC (erythrapheresis), which is currently the safest and most efficient method. However, it is costly, complicated, and cannot be implemented everywhere, nor is it suitable for all patients. Manual exchange transfusions combine one or more manual phlebotomies with a PRBC transfusion. At the Reference Center of Sickle Cell Disease, we set up a continuous method of manual exchange transfusion that is feasible for all hospital settings, demands no specific equipment, and is widely applicable. In terms of HbS decrease, stroke prevention, and iron overload prevention, this method showed comparable efficiency to erythrapheresis. In cases where erythrapheresis is not available, this method can be a good alternative for patients and care centers.