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 et al. 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 et al. 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 (Online ahead of print). doi: 10.1111/bjh.19674.

Successful treatment of severe hepatic impairment in erythropoietic protoporphyria: A case report and review of literature.

BACKGROUND: Erythropoietic protoporphyria (EPP) is a rare genetic disorder stemming from ferrochelatase gene mutations, which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes, skin, bone marrow and liver. Although porphyria-related severe liver damage is rare, its consequences can be severe with limited treatment options. CASE SUMMARY: This case study highlights a successful intervention for a 35-year-old male with EPP-related liver impairment, employing a combination of red blood cell (RBC) exchange and therapeutic plasma exchange (TPE). The patient experienced significant symptom relief and a decrease in bilirubin levels following multiple PE sessions and an RBC exchange. CONCLUSION: The findings suggest that this combined approach holds promise for managing severe hepatic impairment in EPP.

Jk(a) (Kidd-A) Variant in a Sickle Cell Disease Patient.

Sickle cell disease (SCD) is a chronic and prevalent hemoglobin disorder with various manifestations and complications depending on the organs involved. Red cell transfusion either simple or exchange is crucial due to its prophylactic and therapeutic roles. We present a case showing serologic discrepancy between the red cells phenotype and the developed alloantibodies to emphasize the crucial role of molecular testing in SCD patients requiring chronic blood transfusion.

The first reported use of red blood cell exchange to treat hemoglobin Evans with secondary methemoglobinemia.

This manuscript describes a novel approach for treating patients with long-term sequelae from hemoglobin Evans (Hb Evans). After instituting conservative therapies for approximately 2 years, our patient's symptoms continually worsened. Therefore, we performed red blood cell exchange (RBCx) to reduce his Hb Evans percentage and his co-existing elevation of methemoglobin. Our assumptions of clinical benefit were based on our collective experience performing RBCx for patients with sickle cell disease. After the first exchange, pre- and post-laboratory results supported our approach and the patient experienced marked improvement in his clinical signs and symptoms. This report provides preliminary proof of principle for the use of RBCx to treat Hb Evans and other non-Hb S hemoglobinopathies.

A Case of Right Ventricular Failure Secondary to Acute Chest Syndrome Managed With Early Red Cell Exchange Transfusion.

Patients with sickle cell disease are at risk of vaso-occlusive crises including acute chest syndrome (ACS) and pulmonary hypertension. ACS is a life-threatening complication of sickle cell disease and is associated with increased morbidity and mortality. It is known that pulmonary pressures increase during episodes of acute chest syndrome and may lead to acute right ventricular failure leading to increased morbidity and mortality. Given the paucity of randomized controlled trials, the management of ACS and pulmonary hypertension in the setting of a sickle cell crisis largely relies on expert opinion. We present a case of acute chest syndrome complicated by acute right ventricular failure that was managed with prompt red cell exchange transfusion with favorable clinical outcomes.

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Ninth Special Issue.

The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis (TA) in human disease. In the Ninth Edition, the JCA Special Issue Writing Committee has incorporated systematic review and evidence-based approaches in the grading of evidence and categorization of apheresis indications to make recommendations on the use of apheresis in a wide variety of diseases and conditions. This edition has largely maintained the general layout and concept of a fact sheet introduced in the Fourth Edition (2007). Each fact sheet succinctly summarizes the evidence for the use of TA in a specific disease or medical condition. The Ninth Edition of the JCA Special Issue comprises 91 fact sheets and 166 graded and categorized indications. This includes seven new fact sheets, nine new indications on existing fact sheets, and eight changes in the category for existing indications. The Ninth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease.

How do we operate a large monthly red blood cell exchange program.

BACKGROUND: Red blood cell (RBC) exchange for sickle cell disease presents unique difficulties due to RBC phenotyping, complex antibody work-ups, large number of RBC units required, and vascular access considerations, any of which can delay the procedure. Multidisciplinary coordination and systemic processes ensure that monthly appointments remain on schedule. STUDY DESIGN AND METHODS: A high-volume chronic RBC exchange program is described, highlighting the importance of multidisciplinary coordination and process improvement strategies involving initial referral, vascular access, order sets, and allocation of antigen-negative or phenotypically matched RBCs. RESULTS: Approximately 50 outpatient RBC exchanges are performed each month with an 82% kept-appointment rate. Specific factors for program success include open communication across services and improvements to referrals and standardized order sets. CONCLUSION: A combination of multidisciplinary coordination and process improvement can ensure the success of a high volume RBC exchange program. Frequent communication of upcoming appointments between the referring hematologists, the hemapheresis clinic, transfusion service, and interventional radiology is critical. Advance notice to the immunohematology reference lab of upcoming appointments is needed to allow enough time for allocating antigen-negative RBCs. Order sets can be leveraged to standardize and streamline RBC exchanges. Lastly, numerous mechanisms help patients compensate for the cognitive sequelae of stroke.

Analysis of first apheresis services in Ghana-Greater Accra Regional Hospital.

Background: At the combined American Society for Apheresis (ASFA) annual meeting and World Apheresis Association (WAA) Congress in 2014, it was observed that there were significant disparities with regard to the access of apheresis services within and across developing countries, with only few of such facilities available in Africa; notably South Africa and Nigeria. In 2019, Bone Marrow Transplantation (BMT) Unit-Ghana, acquired an apheresis machine. By the collaboration between BMT-Ghana, the Greater Accra Regional Hospital (GARH) and the Ministry of Health (Ghana), apheresis services is now available in Ghana. The aim of this paper is to present an analysis of apheresis services so far in Ghana. Method: A 12-month period from 2019 to 2021 was examined (less the period of the COVID-19 outbreak when the Unit was virtually at a standstill). The electronic database and hard copies of documented activities were analysed. Basic information on demographics and procedure types and counts was used. Results: The retrospective study encompassed data of 43 patients. Two (2) patients came from the West Africa sub-region (Nigeria and Cameroon) with the rest from 6 out of the 14 regions of Ghana (Greater Accra, Western, Central, Eastern, Ashanti, Volta). The essential nature of the apheresis services being the first in Ghana, brought patients as far as 315 km from the hinterlands to the Unit. Ages ranged from 2-52 years with a mean of 16.3 ± 15.3 years. Slightly more females (n = 23, 53%) received services than males (n = 20, 47%). Eighty-six percent (n = 37, 86%) of the patients were sickle cell patients referred to the Unit. Red Blood Cell exchange (RBCx) accounted for 87% (n = 40), of the 46 procedure counts followed by Continuous Mononuclear Cell Collection (CMNC) (n = 4, 9%) and lastly, Therapeutic Plasma Exchange (TPE) (n = 2, 4%). Conclusion: Ghana can now be counted among African countries offering apheresis services and the GARH is acknowledged as the only hospital in the country with this facility, thus improving patient care significantly.

Transfusion support for patients with sickle cell disease.

Red blood cell (RBC) transfusion is an essential treatment for many patients with sickle cell disease (SCD), whose RBCs express hemoglobin S (HbS), a mutated form of hemoglobin A (HbA). Transfusion goals include increasing blood oxygen carrying capacity and decreasing the relative amount of HbS to HbA to mitigate vaso-occlusion in small blood vessels. In situations where correction of severe anemia and reduction in HbS may be achieved without removal of RBCs, simple transfusion may be utilized. Partial manual RBC exchange, which removes blood containing HbS by phlebotomy and replaces with donor blood transfusion sequentially allows for larger changes in the ratio of HbS to HbA when compared to simple transfusion. Automated RBC exchange by apheresis is useful in situations where a rapid and drastic HbS reduction is indicated. Vascular access is an important consideration for transfusion. Although peripheral access may be sufficient, central venous catheters and implantable venous access devices may be necessary for adequate access over time. Blood bank considerations include adequate RBC antigen matching to mitigate the risk of RBC alloimmunization, of which patients with SCD are at risk of developing. Transfusion may be utilized in efforts to intervene in the evolution of potentially life-threatening complications of SCD such as acute stroke, severe acute anemia and acute chest syndrome. Transfusion is also useful in several non-acute settings, such as stroke prevention, pregnancy, pre-surgery, and transfusion support for curative therapies. Individualized treatment plans are an essential component of patient care. Continuous evaluation of clinical indications and evolution of guidelines will continue to optimize care for patients with SCD.

Role of automated red blood cell exchange in the treatment of aluminum phosphide poisoning: A case report and review of the literature.

Aluminum phosphide (AIP) is a fumigant commonly used in agricultural areas. AIP is frequently misused for suicidal purposes because it is easily accessible. AIP poisoning causes severe metabolic acidosis, resistant hypotension, acute respiratory distress syndrome, and multiorgan failure with cardiogenic shock. Despite supportive management and intensive care, most patients die following AIP ingestion because there is no specific antidote. In this case report we present a 15-year-old female who presented with vomiting, coma and epigastric pain. She developed resistant metabolic acidosis and hypotension due to AIP poisoning. Although supportive treatment did not result in clinical improvement, she was successfully treated with automated red blood cell exchange. Automated red blood cell exchange is a procedure which is used to exchange the patient erythrocyte mass with donor red blood cell. Although automated red blood cell exchange is a preferred treatment method in the complications of sickle cell anemia, some blood diseases and infectious diseases such as malaria and babesiosis, there is little information about its use in poisoning. To the best of our knowledge, this is the first child with AIP poisoning who was treated with automated red blood cell exchange.

Red blood cell exchange in children with sickle cell disease.

The aim of our study was to assess the efficacy of red blood cell exchange (RBCx) using a Spectra Optia® automated apheresis system in children with sickle cell disease (SCD). We used automated RBCx to treat acute and chronic complications in 75 children with SCD who had a median age of 10 years [7-13]. We analyzed 649 RBCx sessions. Peripheral venous access was limited in a number of the children, and thus a femoral double-lumen central venous catheter was required. We recommend heparin locking with 500 units in each lumen of the catheter. To prevent complications, we ensured that all patients had achieved a post-RCE HbS level of < 30%. For chronic transfusion, with a post-RCE Hb level of approximately 10-11 g/dL, a blood exchange volume of ≥ 32 mL/kg, and an interval between each RBCx procedure of ≤ 30 days, the residual HbS level was maintained below 30%. For acute transfusion, a post-exchange Hb level ≥ 10 g/dL (p < 0.001) and a total exchange volume ≥ 35 mL/kg (p = 0.001) were the best way to reduce HbS to < 30%. AUC was 0.84. Our results show that erythrocytapheresis was useful and safe for children with SCD.

A Fresh Breath of Oxygen: Red Blood Cell Exchange Transfusion in Sickle Cell and COVID-19.

Red blood cell exchange transfusion may be beneficial and should be considered in the early management of patients with sickle cell disease and COVID-19 to prevent the need for intubation and intensive care unit admission due to respiratory distress.

Acute Liver Failure From Sickle Cell Hepatopathy Treated With Exchange Transfusion.

Sickle cell disease (SCD) is a qualitative hemoglobinopathy that can cause widespread sickling and vaso-occlusive events in all organ systems. Sickle cell hepatopathy is an umbrella term for various acute and chronic pathologies of the liver as a result of sickling in SCD patients. We present below the case of a 49-year-old woman who had an acute liver failure in the setting of a hepatic crisis with recovery after exchange transfusion. Hepatic involvement in SCD may be life-threatening. Understanding the etiology and severity of hepatic involvement by sickling is necessary for appropriate treatment.

Apheresis practice patterns in the United States of America: Analysis of a market claims database.

INTRODUCTION: Indications for apheresis procedures are expanding; however, the evidence for many is low quality. A better understanding of apheresis patterns in the United States is needed to better plan prospective research studies. METHODS: Data from January 1, 2013, to September 30, 2015, were analyzed from the IBM MarketScan Research Databases of de-identified health insurance claims data of several million enrollees at all levels of care from large employers and health plans across the United States. Apheresis procedures were identified by International Classification of Diseases, Ninth version (ICD-9) and Current Procedure Terminology (CPT) codes. RESULTS: Combining inpatients and outpatients, 18 706 patients underwent 70 247 procedures. The patients were 52.7% female, 5.1% <18 years, and 55.9% inpatient, while the procedures were 49.5% female, 5.7% <18 years, and 19.8% inpatient. For each apheresis modality, the percent of patients treated and procedures performed, respectively, are plasmapheresis 36.4% and 42.5%, autologous harvest of stem cells 22.8% and 10.7%, plateletpheresis 11.1% and 3.5%, allogeneic harvest of stem cells 8.2% and 2.5%, photopheresis 5.4% and 24.4%, erythrocytapheresis 3.8% and 4.7%, leukopheresis 2.0% and 0.7%, immunoadsorption 1.4% and 0.4%, extracorporeal selective adsorption/filtration and plasma reinfusion 1.0% and 3.6%, and other 21.6% and 6.9%. A wide variety of diagnoses were treated; however, analysis of the diagnoses suggests the procedure codes may not always reflect an apheresis procedure. CONCLUSION: This study describes the landscape of apheresis in the United States, but may overestimate some procedures based on linked diagnosis codes. Direct measures of apheresis procedures are needed to plan future research studies.

Red Blood Cell Exchange in a Patient With Extramedullary Hematopoiesis and Cor Pulmonale Secondary to Beta Thalassemia.

RATIONALE: Beta thalassemia is a congenital defect in the production of the beta globin chain. Patients with beta thalassemia major will have higher levels of hemoglobin F (HbF), which is suboptimal in releasing oxygen to tissue. Herein, we describe the use of red blood cell (RBC) exchange transfusion, a therapy typically used in sickle cell patients, in the management of a patient with beta thalassemia with extensive extramedullary hematopoiesis and elevated levels of HbF. Patient concerns: A 34-year-old male of mixed African American and Southeast Asian descent with a known history of beta thalassemia major presented with progressive dyspnea on exertion with marked fatigue. Diagnoses: The patient was transferred to our facility for management of acute hypoxemic, hypercapnic respiratory failure associated with cor pulmonale. INTERVENTIONS: The patient was initially managed with non-invasive positive pressure support ventilation (NIPPV) and intravenous diuresis. Hydroxyurea and epoprostenol nebulization were added to his treatment regimen; however, he progressively became more unstable, necessitating inotropic support. With extramedullary hematopoiesis leading to mass-like effect on critical organs and very high HbF (96%) thought to contribute to his presentation, red blood cell exchange transfusion was initiated once the blood pressure stabilized. OUTCOMES: The patient clinically improved, and was discharged home within a week on supplemental oxygen by nasal cannula and long-term red blood cell exchange. LESSONS: We postulated that significantly elevated HbF contributed to the patient's chronic hypoxia and subsequent respiratory complications. Based on the patient's clinical improvement following the intervention, we believe that RBC exchange transfusion could be considered in the management of beta thalassemia patients with significantly elevated levels of HbF.

Severe hemolysis and vaso-occlusive crisis due to COVID-19 infection in a sickle cell disease patient improved after red blood cell exchange.

Even though most data suggest favorable outcome in patients with SCD and COVID-19 infection, close monitoring remains essential as acute complication may develop unexpectedly. Offering RBC exchange early in the course of infection might improve prognosis.

Priming With Red Blood Cells Allows Red Blood Cell Exchange for Sickle Cell Disease in Low-Weight Children.

Red blood cell exchanges are frequently used to treat and prevent cerebrovascular complications in patients with sickle cell anemia (SCA). However, the low weight of young children represents serious concerns for this procedure. The Spectra Optia device can perform automatic priming using red blood cells (RBCs) (RCE/RBC-primed) which could allow RBC exchanges (RCE) to be performed in young children without hypovolemic complications, but this method requires evaluation. We prospectively analyzed the clinical safety of the RCE/RBC-primed procedure in 12 SCA low-weight children under either a chronic RCE program or emergency treatment over 65 sessions. We monitored grade 2 adverse events (AEs) such as a decrease in blood pressure, increase in heart rate, fainting sensation, or transfusion reactions and identified the critical times during the sessions in which AEs could occur. Post-apheresis hematocrit (Hct) and a fraction of cell remaining (FCR) values were compared to the expected values. We also compared the impact of automatic RCE (n = 7) vs. RCE/RBC-primed (n = 8) on blood viscosity and RBC rheology. A low incidence of complications was observed in the 65 RCE sessions with only seven episodes of transient grade 2 AEs. Post-apheresis Hct and FCR reached expected values with the RCE/RBC-primed method. Both the automatic and priming procedures improved RBC deformability and decreased the sickling tendency during deoxygenation. Blood rheological features improved in both RCE/RBC-primed and automatic RCE without priming conditions. The RCE/RBC-primed procedure provides blood rheological benefits, and is safe and efficient to treat, notably in young children with SCA in prophylactic programs or curatively when a SCA complication occurs.

The role of red blood cell exchange in sickle cell disease in patient with COVID-19 infection and pulmonary infiltrates.

Due to the overlap between ACS and COVID-19 pneumonia, we recommend close monitoring for those patients and offering them RBC exchange early in the course of the disease to avoid clinical deterioration.

Red blood cell exchange in sickle cell disease patient with multiple alloantibodies.

There are several reports in medical literature about Red Cell Exchange (RCE) being routinely performed pre-operatively in sickle cell disease patients to provide immediate decrease in HbS concentration and prevent post-operative complications. We would like to present one such case of SCD who also had multiple allo-antibodies and had to undergo hemi-arthroplasty for avascular necrosis of head femur. Grouping and antibody screening was performed using column agglutination technique. 3-cell and 11- cell panel were used for antibody screening and identification, respectively. Automated RBC exchange was performed on apheresis machine Com. Tec using the standard PL1 kit (Fresenius Kabi, Germany). Multiple (anti-c, E) allo-antibodies were identified and successful pre-operative RCE was done with corresponding antigen-negative AHG compatible RBC units. Single RCE procedure reduced HbS concentration from 65% to 25%. The patient underwent uneventful hemi-arthroplasty and was discharged on post-operative day-7. Patient is on regular follow-up and continues to do well two months after the day of surgery. This is possibly the first case report from India, which illustrates successful automated RCE in a SCD patient with alloimmunization.