Red Blood Cell Exchange Research Articles

Quality of life in people with sickle cell disease treated with automated red blood cell exchange.

This review aims to explore the impact of sickle cell disease (SCD) on patients' quality of life (QoL) and the effectiveness of different transfusion modalities, particularly automated red blood cell exchange (aRBCX), in managing the factors that impact QoL. A systematic search was performed in PubMed to retrieve articles with data on QoL in SCD patients treated with aRBCX during the last 20 years. A targeted search for medical guidelines and a free search were added. When assessing the impact of the transfusion modality on the QoL of patients with SCD, some studies indicated an improvement in health-related QoL when using aRBCX while others reported no differences. The benefits of aRBCX include a decrease in length of hospital stay, pain-related hospitalizations and procedure time. The drawbacks of aRBCX were also identified, including an increased number of procedure-related complications (despite the overall number of complications showing no significant differences) and a more complex vascular access. Chronic red blood cell exchange favours psychosocial factors such as anxiety and social functioning, but the impact of using aRBCX in these parameters is not determined yet. aRBCX, known to be an efficient procedure to manage SCD, appears to be promising in improving patients' QoL. However, more comprehensive studies incorporating patient-reported outcomes are needed to fully understand the impact of different transfusion modalities on QoL in SCD patients. An integrated care approach, including psychological support and pain management, may further enhance QoL.

Hematocrits of red blood cell units increase during storage due to changes in mean corpuscular volume, impacting outcomes of red cell exchange procedures

Abstract Introduction Hematocrits of red blood cell (RBC) units are generally not measured by blood centers or transfusion services. Red blood cell exchange (RCE) is commonly used to treat sickle cell disease, and a key parameter needed in RCE is the average hematocrit of RBC units. However, there is no consensus on the average hematocrit to use or how it is affected by storage. The goal of our study is to determine the average hematocrit of RBC units by measuring hematologic parameters RBC units during storage and performing statistical modeling of hematocrit changes over time. Methods We sampled aliquots of leukoreduced RBC units in AS-1 (n=10) using the Terumo TSCD-II Sterile Tubing Welder to sterilely collect ~15mL of RBCs in transfer-pack containers. Aliquots were collected weekly from Day 7 to Day 42 of storage. Complete blood counts were performed using the Sysmex XN-3100 Hematology Analyzer. We statistically modeled changes in RBC units during storage by utilizing Bayesian multilevel linear regression models, allowing for capturing effects at the individual RBC-unit level. Posterior distributions of model parameters were estimated and processed using Stan and the rethinking and rstan packages in R. Posterior distributions are summarized with posterior means and 95%-credible intervals in parentheses. Model selection was done by calculating Akaike weights using the widely applicable information criterion (WAIC) to signify statistical support for each model. Results Multilevel linear regression models of hematologic parameters as a function of storage time were developed. Hematocrits increased with a posterior mean of 1.4% (0.98–1.75%) per week of storage, with posterior mean hematocrits of: 61.9% (60.2–63.6%) on Day 7, 63.9% (61.5–66.4%) on Day 21, and 66.0% (62.8–69.7%) on Day 42. These results indicate that a single average cannot be used for RBC units since hematocrits dramatically increase during storage. Mean corpuscular volume (MCV) increased with a posterior mean of 2.31 fL (1.4–3.71) per week whereas hemoglobin remained stable with a posterior mean increase of 0.008 g/dL (-0.016–0.031) per week. Using these results, we subsequently developed a multilevel regression model of hematocrit as a function of MCV. The model using MCV (WAIC=100.2, weight=100%) was favored over the model using time (WAIC=120.0, weight=0%), suggesting that changes in MCV are driving the increasing hematocrits. Conclusion Our study is the first to leverage Bayesian statistics to carefully quantify the average hematocrits of RBC units during storage time and to demonstrate that increasing hematocrits are caused by changes in MCV. These results indicate that transfusion protocols must consider the age of RBC units for RCE calculations, and provisions (e.g., requiring use of fresh units for RCE) may help minimize the variability in post-exchange testing. Moreover, our findings suggest that transfusion services should regularly measure hematocrits of RBC units in their inventory.

Evaluating Thalassemia Awareness in Academic Environments: A Comparative Study among Medical and Non-medical Students

Thalassemia is a very common hemoglobinopathy that is present worldwide. When one of alpha and beta chains is not present in hemoglobin or is present in lesser amounts it causes thalassemia. 9 million population of Pakistan are carriers of thalassemia. 5% of the world’s cases of thalassemia are from Pakistan. This study is designed to check thalassemia awareness among medical and non-medical students. This was a cross-sectional study. 112 students were taken to answer the questionnaire through a non-random sampling technique. Out of the total participants, 57% were female respondents and 43% were male. Similarly, medical students were 58% and non-medical students were 42%. Of these, 81% medical and 57.1% of non-medical students knew that thalassemia is a genetic disorder. 64.5% of medical and 47.8% of non-medical students thought that there was a cure for thalassemia. Medical and non-medical students of 92% and 63.6% respectively, knew that thalassemia is treated with red blood cell exchange. 54.8% medical and 25% non-medical students knew that thalassemia is not contagious. It is concluded that medical students have more awareness of thalassemia than non-medical students. Medical students have more knowledge about the treatment and contagiousness of this disorder.

Do Babesia microti Hosts Share a Blood Group System Gene Ortholog, Which Could Generate an Erythrocyte Antigen That Is Essential for Parasite Invasion?

The United States of America (US) has the highest annual number of human babesiosis cases caused by Babesia microti (Bm). Babesia, like malaria-causing Plasmodium, are protozoan parasites that live within red blood cells (RBCs). Both infectious diseases can be associated with hemolysis and organ damage, which can be fatal. Since babesiosis was made a nationally notifiable condition by the Centers for Disease Control and Prevention (CDC) in January 2011, human cases have increased, and drug-resistant strains have been identified. Both the Bm ligand(s) and RBC receptor(s) needed for invasion are unknown, partly because of the difficulty of developing a continuous in vitro culture system. Invasion pathways are relevant for therapies (e.g., RBC exchange) and vaccines. We hypothesize that there is at least one RBC surface antigen that is essential for Bm invasion and that all Bm hosts express this. Because most RBC surface antigens that impact Plasmodium invasion are in human blood group (hBG) systems, which are generated by 51 genes, they were the focus of this study. More than 600 animals with at least one hBG system gene ortholog were identified using the National Center for Biotechnology Information (NCBI) command-line tools. Google Scholar searches were performed to determine which of these animals are susceptible to Bm infection. The literature review revealed 28 Bm non-human hosts (NHH). For 5/51 (9.8%) hBG system genes (e.g., RhD), no NHH had orthologs. This means that RhD is unlikely to be an essential receptor for invasion. For 24/51 (47.1%) hBG system genes, NHH had 4-27 orthologs. For the ABO gene, 15/28 NHH had an ortholog, meaning that this gene is also unlikely to generate an RBC antigen, which is essential for Bm invasion. Our prior research showed that persons with blood type A, B, AB, O, RhD+, and RhD- can all be infected with Bm, supporting our current study's predictions. For 22/51 (43.1%) hBG system genes, orthologs were found in all 28 NHH. Nineteen (37.3%) of these genes encode RBC surface proteins, meaning they are good candidates for generating a receptor needed for Bm invasion. In vitro cultures of Bm, experimental Bm infection of transgenic mice (e.g., a CD44 KO strain), and analyses of Bm patients can reveal further clues as to which RBC antigens may be essential for invasion.

Iron metabolism in sickle cell disease patients undergoing chronic red blood cell exchange: A delicate homeostasis in balance.

Sickle cell disease (SCD) is an inherited haemoglobinopathy associated with significant morbidity and mortality. Automated red blood cell exchange (aRCE) plays a key role in managing SCD, eliciting both therapeutic and prophylactic effects. The ideal post-apheresis Ht target for chronic aRCE treatment is not yet unanimously recognized, as well as iron homeostasis can be different among patients. Ross etal. reported their experience on the chronic management of SCD patients undergoing aRCE with a final post-exchange Ht higher than the value commonly adopted, analysing red blood cell transfusion requirements and iron-related outcomes in the study population. Commentary on: Ross etal. Automated red blood cell exchange with a post-procedure haematocrit targeted at 34% in the chronic management of sickle cell disease. Br J Haematol 2024; 205:1556-1564.

Automated red blood cell exchange with a post-procedure haematocrit targeted at 34% in the chronic management of sickle cell disease.

Optimal targets for red blood cell exchange (RCE) are not well defined in the chronic management of sickle cell disease. We analysed transfusion requirements and iron-related outcomes in 101 patients on chronic RCE with a post-procedure haematocrit (Ht) targeted at 34%, which is higher than typically used. A majority were of HbSS/HbSβ0 genotype (n = 72) and enrolled for neurological complications (n = 53). Fifty patients had a positive Ht balance with RCE (>2% mean increase from pre-procedure level), while 43 patients maintained a neutral balance. The first group required fewer red blood cell units/year (65 vs. 80, p < 0.001), but a significant proportion were iron overloaded based on R2* with liver MRI (32% vs. none performed) and prescription of iron chelation (52% vs. 0%, p < 0.001, after a median of 19 months). The second group was more likely to receive iron supplementation (6% vs. 56%, p < 0.001). Chronic automated RCE with a post-procedure Ht targeted at 34% is not iron-neutral, and personalized Ht goals may be more appropriate in certain settings. This higher target should be compared with a lower Ht strategy in individuals with similar baseline red cell volumes to assess iron homeostasis and blood product requirements.

A multi-institutional survey of apheresis services among institutions in the United States.

Apheresis practices in the United States (US) have not been comprehensively characterized to date. This study aimed to address this gap by evaluating apheresis therapy through a national survey. A multi-institutional survey was conducted between April and July 2023. The survey, comprising 54 questions, focused on institutional demographics, procedures, equipment, staffing, training, and impacts of the Coronavirus Disease 2019 (COVID-19) pandemic. Responses from 22 institutions, primarily academic medical centers, were analyzed. Therapeutic plasma exchange (TPE) was the most common procedure, followed by hematopoietic progenitor cell collection (HPC-A) and red blood cell exchange (RCE). CAR-T cell collections were widespread, with some institutions supporting over 30 protocols concurrently. Most sites used the Spectra Optia Apheresis System, were managed by a transfusion medicine service, and employed internal apheresis providers. Insufficient staffing levels, exacerbated by the COVID-19 pandemic, were common and most often addressed using overtime. The survey highlighted the ubiquity of TPE, expanding cellular collections and staffing challenges. The role of apheresis in supporting cellular therapy, particularly in newly developing cell and gene therapies and clinical trials, was evident. Staffing issues during the pandemic emphasized the need for innovative recruitment strategies. This nationwide survey provides the most comprehensive analysis to date of apheresis practices in large US academic centers.

The enigma of sickle cell hepatopathy: Pathophysiology, clinical manifestations and therapy.

Sickle cell disease (SCD) is one of the most common genetic disorders in the world predominantly affecting economically disadvantaged populations. There is a notable discrepancy between the growing adult SCD population and available diagnostic and therapeutic interventions for SCD. Sickle cell hepatopathy (SCH) is an all-inclusive term to describe the acute and chronic liver manifestations of SCD. The pathophysiology of SCH follows no defined pattern or sequence that poses challenges to clinicians and researchers alike. Evidence is lacking for this underreported disease at various levels from diagnostic to therapeutic options. This paper reviews the basic pathophysiology, clinical features, biochemical and radiological findings of various SCH manifestations and outlines the management of each condition. Old and new therapy options in SCD including hydroxyurea, red blood cell exchange transfusion, ursodeoxycholic acid, voxelotor, l-glutamine and crizanlizumab have been reviewed to investigate the role of these options in treating SCH. The role of liver transplant, haematopoietic stem cell transplant and gene therapy in SCH patients have been reviewed.

Neonatal and Obstetrical Outcomes of Pregnancies Complicated by Alloimmunization.

Despite advances in the prevention of rhesus (Rh)(D) alloimmunization, alloantibodies to Rh(D) and non-Rh(D) red blood cell antigens continue to be detected in ∼4% of US pregnancies and can result in hemolytic disease of the fetus and newborn (HDFN). Recent reports on HDFN lack granularity and are unable to provide antibody-specific outcomes. The objective of this study was to calculate the frequency of alloimmunization in our large hospital system and summarize the outcomes based on antibody specificity, titer, and other clinical factors. We identified all births in a 6-year period after a positive red blood cell antibody screen result during pregnancy and summarized their characteristics and outcomes. A total of 707 neonates were born after a positive maternal antibody screen result (3.0/1000 live births). In 31 (4%), the positive screen result was due to rhesus immune globulin alone. Of the 676 neonates exposed to alloantibodies, the direct antibody test (DAT) result was positive, showing antigen-positivity and evidence of HDFN in 37% of those tested. Neonatal disease was most severe with DAT-positive anti-Rh antibodies (c, C, D, e, E). All neonatal red blood cell transfusions (15) and exchange transfusions (6) were due to anti-Rh alloimmunization. No neonates born to mothers with anti-M, anti-S, anti-Duffy, anti-Kidd A, or anti-Lewis required NICU admission for hyperbilirubinemia or transfusion. Alloimmunization to Rh-group antibodies continues to cause a majority of the severe HDFN cases in our hospital system. In neonates born to alloimmunized mothers, a positive DAT result revealing antigen-positivity is the best predictor of anemia and hyperbilirubinemia.

The Optimized Parameters of Red Blood Cell Exchange by Apheresis in Transfusion-Dependent Thalassemia, a Small Case Series

The Optimized Parameters of Red Blood Cell Exchange by Apheresis in Transfusion-Dependent Thalassemia, a Small Case Series

Prevention of potential delayed hemolytic transfusion reaction in two sickle cell patients using intravenous immunoglobulins and steroids before and after red blood cell exchange with antigen positive units and review literature

Emergent Red Blood Cell (RBC) exchange is indicated in sickle cell disease (SCD) patients with severe acute chest syndrome. However, fully matched RBC units may not be available for patients with multiple RBC antibodies. Intravenous immunoglobulin (IVIG) and steroids were reported for preventing potential delayed hemolytic transfusion reaction (HTR) in simple transfusion of antigen-positive RBCs. We investigated the efficacy and safety of IVIG and steroids in two SCD patients presented with acute chest syndrome receiving RBC exchange with multiple incompatible units. The first patient had multiple historical alloantibodies, including anti-Jsb, although none of them were reactive. IVIG (1 g/kg) was given before and after RBC exchange with methylprednisolone (500 mg IV) one hour before exchange. Her sickle hemoglobin (HbS) was reduced from 89.4% to 17.4% after the exchange with five Jsb-positive units. The patient improved clinically without acute or delayed hemolysis. The second patient had reactive anti-Jsb on two different admissions 18 months apart. Only one of the sixteen units used in the exchanges was Jsb negative. He received the same IVIG regimen during both admissions but 100 mg IV hydrocortisone instead of methylprednisolone. His HbS was reduced from 63.4% to 22.4% after the first exchange. Significant clinical improvements were achieved after both exchanges. No delayed HTR was observed. Our experience of these two patients suggested that IVIG and steroids may be used in preventing potential delayed HTR in some SCD patients with rare antibodies receiving large amounts of antigen-positive RBC products.

Utility of hemoglobin electrophoresis to distinguish between severe delayed hemolytic transfusion reaction versus hyperhemolysis syndrome

Delayed hemolytic transfusion reaction (DHTR) and hyperhemolysis syndrome (HHS) are both complications of red blood cell transfusions in patients with sickle cell disease.Clinically, both present with hemolysis and can be difficult to differentiate. Hemoglobin electrophoresis may aid in the diagnosis. Herein we describe a case in which a patient with hemoglobin SC disease presented with features of severe hemolysis several days after initiation of red blood cell exchange. Increase in reticulocyte count and complete absence of hemoglobin A on electrophoresis during this event supported the diagnosis of severe DHTR, indicating a rapid and selective destruction of the transfused red blood cells. Ability to interpret the hemoglobin electrophoresis can help clinicians distinguish between these two severe transfusion complications in patients living with sickle cell disease. It is important to identify the presence or absence of concomitant HHS, as patients with HHS tend to have a worse prognosis and there is a higher rate of recurrence of HHS with subsequent transfusions. Accurate diagnosis can lead to prompt management and decrease morbidity and mortality.

Using 31P NMR to quantify methyl-alpha cyclodextrin-induced lipid exchange and lipid asymmetry of red blood cells

Using 31P NMR to quantify methyl-alpha cyclodextrin-induced lipid exchange and lipid asymmetry of red blood cells

Managing sickle cell disease and related complications in pregnancy: results of an international Delphi panel

AbstractData to guide evidence-based management of pregnant people with sickle cell disease (SCD) are limited. This international Delphi panel aimed to identify consensus among multidisciplinary experts for SCD management during pregnancy. The 2-round Delphi process used questionnaires exploring 7 topics (antenatal care, hydroxyurea use, transfusion, prevention of complications, treatment of complications, delivery and follow-up, and bottlenecks and knowledge gaps) developed by a steering committee. Thirteen panelists (hematologists, physiologists, obstetricians, maternal fetal medicine, and transfusion medicine physicians) from the United States, the United Kingdom, Turkey, and France completed the first survey; 12 panelists completed the second round. Anonymized responses were collected and summarized by a contract research organization (Akkodis Belgium). Consensus and strong consensus were predefined as 75% to 90% (9-10 of 12) and >90% (≥11 of 12) of panelists, respectively, agreeing or disagreeing on a response to a predefined clinical scenario or statement. In several areas of SCD management, consensus was achieved: experts recommended performing at least monthly multidisciplinary antenatal follow-up, administering prophylactic aspirin for preeclampsia prevention between gestational weeks 12 and 36, initiating prophylactic transfusion therapy in certain cases, or choosing automated red blood cell exchange over other transfusion methods for patients with iron overload or severe acute chest syndrome. No consensus was reached on several topics including the prophylactic aspirin dose, indications for starting infection prophylaxis, routine use of prophylactic transfusions, or use of prophylactic transfusions for preventing fetal complications. These recommendations could inform clinical care for patients with SCD who are pregnant in the absence of large clinical trials involving this population; the identified knowledge gaps can orient future research.

A Study of Red Cell Exchange in Patients of Sickle Cell Disease at a Tertiary Care Hospital of South Gujarat, India

ABSTRACT Introduction: Sickle cell disease (SCD) is an inherited single-gene autosomal recessive disorder. Red cell exchange (RCE) refers to the removal of diseased red blood cells (RBCs) in exchange for healthy donor RBCs. In the present study, RCE was performed in patients with SCD with different crises such as acute chest syndrome, vaso-occlusive crisis, and sequestration crisis. The study was conducted to determine the clinical efficacy of RCE in SCD and to study the clinical profile of the study population. The study will help generate data to support and improve existing knowledge on the clinical efficacy of RCE in SCD. Methods: A prospective observational study of RBC exchange in patients of SCD with the acute crisis was conducted at a tertiary care hospital in South Gujarat including the effect of RCE as a treatment modality in patients of SCD. The study was conducted following guidelines given by the American Society for Apheresis. Results: This study was conducted among 8 patients (7 female and 1 male) with varying age groups from 10 to 29 years. Clinical findings of patients, pulse rate, respiratory rate, and oxygen saturation got improved after the procedure. There was an improvement in PaO2 from 51.63 to 73 after the procedure. There was a decrease in mean sickle hemoglobin (Hb S) level (from 68.452% to 16.438% after procedure) and Hb F level (from 11.938% to 3.063% after procedure) with an increase in Hb A level (from 13.862% to 70.152% after procedure). Conclusion: In the present study, automated RCE procedures in patients with SCD were well tolerated and effective for both acute and chronic complications of SCD in terms of relieving the symptoms by reducing the Hb S level postprocedures.

Manual red cell exchange (RBCXm) in acute sickle cell crisis: A feasible modality in resource limited settings

This short communication highlights the significance of manual red blood cell exchange (RBCXm) procedure which is an emergency procedure for patients of sickle cell disease (SCD) in sickle cell crisis . To the best of our knowledge there is no standardised technique for performing RBCXm meanwhile, an automated method using an apheresis machine is either unavailable or is unaffordable. As a result, patients end up receiving top up red cell transfusions further associated with risk of iron overload, hyper viscosity and sometimes volume overload overshadowing the benefits of red cell exchange. Therefore, we developed a protocol for manual red cell exchange (RBCXm) and performed it in a patient with painful sickle cell crisis who had cost constraints for automated red cell exchange (RBCXa) procedure. RBCXm provided symptomatic relief to our patient with adequate sickle cell reduction. This RBCXm protocol may be useful in low income (LIC) and low middle income countries (LMIC) where patients have recurrent episodes of sickle cell crisis and there is unavailability or unaffordability for RBCXa using an apheresis equipment.

Management of COVID-19 in a Sickle Cell Anaemia Patient: A Case Report

BACKGROUND: COVID-19 has had a significant impact on global healthcare systems, causing severe cases with acute respiratory distress syndrome, multi-organ failure, and death. Survivors of the virus may also experience long-term health problems. Sickle cell disease (SCD), an inherited red blood cell disorder, is characterized by chronic hemolytic anemia, vascular occlusion, and organ deterioration. SCD primarily affects African Americans, and common symptoms include acute and chronic pain, as well as severe conditions like acute chest syndrome and stroke. METHODS: We present the case of a 19-year-old female with sickle cell anemia who presented with symptoms of illness, jaundice, and no fever after receiving intravenous paracetamol. They were stable overall, with a Glasgow Coma Scale (GCS) score of 15/15 and no abnormal chest sounds. The patient had red, soft stools for two days and reduced bowel movements. Bowel sounds were normal, and they had sufficient urine output but experienced lower limb edema without joint swelling. Blood pressure was 107/62 mm Hg, mean arterial pressure was 72 mm Hg, pulse rate was 104 beats per minute, respiratory rate was 21 breaths per minute, and random blood glucose level was 102.5 mg/dL. The patient tested positive for COVID-19 via Polymerase Chain Reaction (PCR) testing. Laboratory results revealed low hemoglobin (6.8 g/dL), red blood cell count (2.16 x 10^6/micL), and high white blood cell count (22.4 x 10^3/micL) with 75.4% neutrophils. Hematocrit was 18.5%, mean corpuscular volume (MCV) was 85.7 pictogram/cell, mean corpuscular hemoglobin (MCH) was 31.4, mean corpuscular hemoglobin concentration (MCHC) was 36.7 g/dL, and platelet count was 215/micL. Other laboratory findings included urea (8 mg/dL), total protein (6.8 g/L), total bilirubin (2.15 mg/dL), direct bilirubin (0.98 mg/dL), aspartate aminotransferase (AST) (107 IU/L), alanine aminotransferase (ALT) (30 IU/L), alkaline phosphatase (ALP) (178 IU/L), and C-reactive protein (CRP) (48 mg/L). The patient's management plan involved intravenous dextrose and sodium chloride infusion, ceftriaxone (Samixon®), warfarin as needed, paracetamol as needed, folic acid once daily, vitamin C and zinc supplementation, levofloxacin once daily, Enema (Enemax) as needed, Pantoprazole (pantodac) (40 mg), nebulized hypertonic saline solution three times daily, diclofenac sodium (vortex) (75 mg) twice daily, and rivaroxaban (15 mg) twice daily. The patient received two blood transfusions during their hospital stay, with the first occurring on the first day of admission. CONCLUSION: Sickle cell anemia patients are at a higher risk of severe COVID-19 infection and associated complications. Early detection and treatment of complications are crucial. Blood transfusion, particularly red blood cell exchange transfusion, may be beneficial in managing these patients, reducing respiratory distress and the need for intensive care. However, more research is needed to establish optimal management strategies. This case report provides valuable insights into the presentation and management of sickle cell anemia patients with COVID-19. Clinicians should consider the examination findings and laboratory investigations for diagnosis and management. Limitations of this report include the lack of baseline information and long-term follow-up data, emphasizing the need for further research to generalize the findings.

Logistics, risks, and benefits of automated red blood cell exchange for patients with sickle cell disease.

Red blood cell (RBC) transfusions treat and prevent severe complications of sickle cell disease (SCD) and can be delivered as a simple or exchange transfusion. During an exchange, some of the patient's abnormal hemoglobin (Hb) S (HbS) RBCs are removed. An apheresis device can accomplish an automated RBC exchange, simultaneously removing patient's RBCs while returning other blood components along with normal RBCs. Automated RBC exchange is therefore an isovolemic transfusion that can efficiently decrease HbS RBCs while limiting iron loading and hyperviscosity. However, specialized equipment, trained personnel, appropriate vascular access, and increased RBC exposure are required compared to simple or manual RBC exchange. Therefore, risks and benefits must be balanced to make individualized decisions for patients with SCD who require transfusion.

Euvolemic automated transfusion to treat severe anemia in sickle cell disease patients at risk of circulatory overload.

Red blood cell (RBC) transfusion remains a major treatment for sickle cell disease (SCD). Patients with SCD have a high prevalence of renal impairment and cardiorespiratory disease, conferring risk of transfusion-associated circulatory overload (TACO). We describe an approach, titled euvolemic automated transfusion (EAT), to transfuse SCD patients with severe anemia who are at risk of TACO. In EAT, plasmapheresis is performed using donor RBCs, rather than albumin or plasma, as replacement fluid. Euvolemia is maintained. A retrospective analysis was conducted of patients with SCD who underwent EAT at our institution over a 10-year period, to evaluate the efficacy and safety of EAT. Eleven SCD patients underwent 109 EAT procedures (1-59 procedures per patient). The median age was 42 years (IQR = [30-49]) and 82% (n = 9) were female. Most (82%; n = 9) patients had severe chronic kidney disease and 55% (n = 6) had heart failure. One (9%) patient had a history of life-threatening TACO. Mean pre- and post-procedure Hct values were 19.8% (SD ± 1.6%) and 29.1% (SD ± 1.4%), respectively. The average Hct increment was 3.2% per RBC unit. Only two EAT-related complications were recorded during the 109 procedures: central line-associated infection and citrate toxicity (muscle cramping). EAT used an average of two RBC units less than that projected for standard automated RBC exchange. Our findings suggest that EAT is safe and effective to treat patients with SCD and severe anemia, who are at risk for TACO. EAT requires fewer RBC units compared to automated RBC exchange.

Hemapheresis Clinic Scheduling Quality Improvement Project

Abstract Introduction/Objective Outpatient (OP) Red blood cell exchange (RCE) therapy is used to treat patients with severe manifestations of sickle cell disease. Like any blood transfusion, a patient must have an active type and screen, drawn 24 to 72 hours prior to scheduled RCE procedure. UIH Hemapheresis clinic performs several types of apheresis procedures treating sickle cell disease, kidney transplant rejection and autoimmune disorders. Outpatient clinic staff perceived that most RCE procedures are scheduled disproportionately between Wednesday through Friday, which creates an unbalanced schedule that is lighter on Monday and Tuesday, and heavier Wednesday through Friday. The study's objective was to determine if there is a significant difference between the number of outpatient procedures scheduled on the different days of the week. If significant differences are identified, processes may be improved to schedule a more balanced OP clinic week. Potential benefits may include a more balanced patient to nursing staff ratios which can improve patient care and more efficient RBC utilization which can ease workload across multiple cross-functional departments such as nursing staff, reference labs and in-house blood bank staff. Methods/Case Report The UIH Epic Hemapheresis Clinic schedule was searched to identify how many total OP procedures, including RCE, were scheduled each day of the week between 01AUG2021 – 31JUL2022. Only OP procedures occurring in complete five-day weeks were included. Weekends and weeks having clinic closures for holidays were excluded Results (if a Case Study enter NA) Our study show that the lowest number of OP RCE (mean 1.4) and total number of OP procedures (mean 2.6) were on Monday. Wednesday is the busiest day with overall averaging 3.5 total procedures (Standard Deviation: 1.56). However, Friday is the most variable, with the largest SD for both RBCX (SD 1.4) and Total number of procedures (SD 1.7). Conclusion Contributing factors to low procedure volume on Monday are likely multifactorial. Some patients may prefer not to come to the hospital on Saturday for type and screen, while some may forget they need labs. Transportation issues are another factor. One intervention the clinic has implemented is automatic appointment call reminders. Staff encourage patients or caregivers to sign up for the EPIC MyChart app which also notifies patients of upcoming appointments. Future studies need to be conducted to address other bottlenecks within the scheduling workflow and to determine ways to optimize patient appointment adherence.