Implementation and early outcomes with Pathogen Reduced Cryoprecipitated Fibrinogen Complex.

OBJECTIVES: Cryoprecipitated antihemophilic factor (cryo) has been used for fibrinogen replacement in actively bleeding patients, dysfibrinogenemia, and hypofibrinogenemia. Cryo has a shelf life of 4 to 6 hours after thawing and a long turnaround time in issuing the product, posing a major limitation of its use. Recently, the US Food and Drug Administration approved Pathogen Reduced Cryoprecipitated Fibrinogen Complex (INTERCEPT Fibrinogen Complex [IFC]) for the treatment of bleeding associated with fibrinogen deficiency, which can be stored at room temperature and has a shelf life of 5 days after thawing. METHODS: We identified locations and specific end users with high cryoprecipitate utilization and waste. We partnered with our blood supplier to use IFC in these locations. We analyzed waste and turnaround time before and after implementation. RESULTS: Operative locations had a waste rate that exceeded nonoperative locations (16.7% vs 3%) and were targeted for IFC implementation. IFC was added to our inventory to replace all cryo orders from adult operating rooms, and waste decreased to 2.2% in these locations. Overall waste of cryoprecipitated products across all locations was reduced from 8.8% to 2.4%. The turnaround time for cryoprecipitated products was reduced by 58% from 30.4 minutes to 14.6 minutes. CONCLUSIONS: There has been a substantial decrease in waste with improved turnaround time after IFC implementation. This has improved blood bank logistics, improved efficiency of patient care, and reduced costly waste.

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.

Mobile calculator application for estimating human erythrocyte antigen frequency in Korea.

OBJECTIVES: This study aimed to establish a comprehensive human erythrocyte antigen (HEA) frequency data set for Koreans. It also sought to develop a mobile app that facilitates the calculation of the frequencies of specific antigen-negative red blood cell units and the average number of units required for antigen typing. METHODS: Human erythrocyte antigen frequencies were compiled from large-scale blood donor data and 5 previous papers. Based on the collected data, we developed a mobile calculator app for HEA frequency and evaluated its usability. RESULTS: Human erythrocyte antigen frequency data for 20 blood group systems, including the ABO, Rh, MNS, Duffy, Kidd, and Diego systems, were established. The app was designed to enable users to select the desired phenotype from a drop-down menu and display the calculated frequency at the bottom. The number of units required for antigen typing to find 1 compatible red blood cell unit was also displayed. Five users participated in app evaluation and rated the functionality and information categories highly. In quizzes prompting users to calculate frequencies using the app, all participants provided correct answers, confirming the app's user-friendly functionality. CONCLUSIONS: This app, which encompasses comprehensive HEA frequency data, is expected to find multiple uses in transfusion medicine, including optimizing blood bank workflow and defining rare blood groups in Korea.

Hospital policy of tranexamic acid to reduce transfusion in major non-cardiac surgery (TRACTION): protocol for a phase IV randomised controlled trial.

INTRODUCTION: Tranexamic acid (TXA) is an inexpensive and widely available medication that reduces blood loss and red blood cell (RBC) transfusion in cardiac and orthopaedic surgeries. While the use of TXA in these surgeries is routine, its efficacy and safety in other surgeries, including oncologic surgeries, with comparable rates of transfusion are uncertain. Our primary objective is to evaluate whether a hospital-level policy implementation of routine TXA use in patients undergoing major non-cardiac surgery reduces RBC transfusion without increasing thrombotic risk. METHODS AND ANALYSIS: A pragmatic, registry-based, blinded, cluster-crossover randomised controlled trial at 10 Canadian sites, enrolling patients undergoing non-cardiac surgeries at high risk for RBC transfusion. Sites are randomised in 4-week intervals to a hospital policy of intraoperative TXA or matching placebo. TXA is administered as 1 g at skin incision, followed by an additional 1 g prior to skin closure. Coprimary outcomes are (1) effectiveness, evaluated as the proportion of patients transfused RBCs during hospital admission and (2) safety, evaluated as the proportion of patients diagnosed with venous thromboembolism within 90 days. Secondary outcomes include: (1) transfusion: number of RBC units transfused (both at a hospital and patient level); (2) safety: in-hospital diagnoses of myocardial infarction, stroke, deep vein thrombosis or pulmonary embolism; (3) clinical: hospital length of stay, intensive care unit admission, hospital survival, 90-day survival and the number of days alive and out of hospital to day 30; and (4) compliance: the proportion of enrolled patients who receive a minimum of one dose of the study intervention. ETHICS AND DISSEMINATION: Institutional research ethics board approval has been obtained at all sites. At the completion of the trial, a plain language summary of the results will be posted on the trial website and distributed in the lay press. Our trial results will be published in a peer-reviewed scientific journal. TRIAL REGISTRATION NUMBER: NCT04803747.

When and why is red blood cell genotyping applicable in transfusion medicine: a systematic review of the literature.

This review aims to provide a better understanding of when and why red blood cell (RBC) genotyping is applicable in transfusion medicine. Articles published within the last 8 years in peer-reviewed journals were reviewed in a systematic manner. RBC genotyping has many applications in transfusion medicine including predicting a patient's antigen profile when serologic methods cannot be used, such as in a recently transfused patient, in the presence of autoantibody, or when serologic reagents are not available. RBC genotyping is used in prenatal care to determine zygosity and guide the administration of Rh immune globulin in pregnant women to prevent hemolytic disease of the fetus and newborn. In donor testing, RBC genotyping is used for resolving ABO/D discrepancies for better donor retention or for identifying donors negative for high-prevalence antigens to increase blood availability and compatibility for patients requiring rare blood. RBC genotyping is helpful to immunohematology reference laboratory staff performing complex antibody workups and is recommended for determining the antigen profiles of patients and prospective donors for accurate matching for C, E, and K in multiply transfused patients. Such testing is also used to determine patients or donors with variant alleles in the Rh blood group system. Information from this testing aides in complex antibody identification as well as sourcing rare allele-matched RBC units. While RBC genotyping is useful in transfusion medicine, there are limitations to its implementation in transfusion services, including test availability, turn-around time, and cost.

Complex Transfusion Management in a Sickle Cell Patient With Anti-Fy3 Alloimmunization: A Case Report.

Fy3 is a high-prevalence red blood cell antigen of the Duffy (Fy) blood group system. Anti-Fy3 antibodies are rare and solely arise in individuals with a Duffy null phenotype (Fy(a-b-)), which is a phenotype that mainly occurs in people of African descent. Clinically, anti-Fy3 antibodies can cause both acute and delayed hemolytic transfusion reactions in adults as well as hemolytic disease in fetuses and newborns. Here, we report the case of a 26-year-old male with sickle cell disease (SCD) and a history of anti-E alloantibodies, who was admitted to the hospital with a vaso-occlusive crisis (VOC) and associated low hemoglobin (Hb) level. For the latter he received one unit of antigen-matched and crossmatch-compatible packed red blood cells (pRBCs) without complications. Ten days later the patient was readmitted with a further VOC and associated low Hb level, again requiring a red cell transfusion. However, no crossmatch-compatible pRBCs could be identified. Laboratory testing demonstrated pan-reactivity with additional reference testing demonstrating the presence of anti-E, anti-Fy3 and anti-Jkb alloantibodies. This case highlights the diagnostic and therapeutic challenges associated with blood transfusion in SCD patients with rare alloimmunization profiles.

A survey assessing knowledge and attitude about blood donation among blood donors in Jordan.

Background: Recruitment of low risk blood donors can be challenging. Efforts should be made to increase the level of awareness and positive attitude towards blood donation. An essential step to achieve this is obtaining comprehensive data about the current situation of awareness, knowledge and attitudes of the population towards blood donation. Methods/materials: The present study was conducted at two blood donation centres in Amman, Jordan, during 2021. A total of 536 whole blood donors were included. Data regarding their demographic characteristics, blood donation history as well as their knowledge and attitudes regarding blood donation were collected by a questionnaire. Results: Four hundred ninety participants (91.4%) were males, whereas only 46 participants (8.6%) were females. Ninety seven subjects (18.1%) were first time donors, whereas 431 subjects (81.9%) had previous donations. The participants' median score in the knowledge section was 19.0 points (range 5-25 points). Based on a cut-off of 15 out of 28: 84% of the participants were knowledgeable. Similarly 97% of the participants had a positive attitude based on a cut-off of 17 out of 32 points. Multivariate analysis revealed that high knowledge score was significantly associated with study major and employment status, whereas a positive attitude was significantly associated with a higher income. More than half of first time donors stated lack of awareness as being the reason for not donating blood before. Conclusion: Measures to improve awareness, knowledge and attitudes towards blood donation should be implemented in order to meet the increasing demand for blood and blood components. Targeted campaigns, correction of some misconceptions and using different motivations are suggested.

Evaluation of activation characteristics of a canine platelet concentrate produced by a commercial double centrifugation system.

Introduction: In veterinary medicine there are few readily available products for platelet transfusion to patients with thrombocytopenia. Commercial tabletop platelet concentrating systems have recently become available to veterinarians, primarily directed towards uses associated with regenerative medicine. These systems could potentially be used to produce fresh concentrated platelets for use in transfusion medicine. This study evaluated the concentration, activation, and sterility of a double centrifugation platelet concentrate (PC) produced by a commercial benchtop system. Methods: Ten healthy dogs were studied. Whole blood was collected and mixed with ACD-A in a 1:7.6 ratio of ACD-A to whole blood. 12 mL of this mixture was processed into PC via single centrifugation, while 60 mL of the anticoagulated whole blood was processed via a commercial double centrifugation system. Both types of PC were evaluated for platelet concentration, CD62P expression with and without thrombin stimulation, and for sterility. Results: Mean platelet count in the double centrifuged PC was 863 ± 352 × 103/µL, with very low white blood cell contamination (median of 0.47 × 103 leukocyte/µL (range 0.15-2.18 × 103/µL)). The double-centrifuged PC had similar baseline activation characteristics (as determined by P-selectin expression) as the single centrifuge PC (0.76% vs. 0.72% unstimulated, 30.5% vs. 34.9% stimulated, p = 0.432). Discussion: The benchtop PC system studied here did not cause activation of platelets during production and produced a sterile product that can be further investigated as a source of fresh platelet concentrates for transfusion purposes.

Peroperative administration of tranexamic acid in Roux-en-Y and one-anastomosis gastric bypass to reduce haemorrhage in patients with morbid obesity: protocol for randomised controlled trial (PATRY trial).

INTRODUCTION: By implementation of Enhanced Recovery After Bariatric Surgery protocols and day-care surgery, early discharge poses a challenge if excessive bleeding occurs after bariatric surgery. Tranexamic acid (TXA) has demonstrated efficacy in other surgical fields and in bariatric pilot studies. This trial aims to assess the efficacy of peroperative administration of TXA in reducing haemorrhage in patients undergoing gastric bypass surgery. METHOD AND ANALYSIS: This is a multicentre, phase III, double-blind randomised controlled trial in six high-volume bariatric centres in the Netherlands. A total of 1524 eligible patients, aged 18 years or older, undergoing primary gastric bypass surgery (either Roux-en-Y gastric bypass or one-anastomosis gastric bypass) will be randomised between TXA and placebo (1:1, variable block, stratified for centre, day-care/overnight stay and type of surgery) after obtaining informed consent (2.5% less haemorrhage, power 80%, 2-sided-α 0.05 and 10% dropout). Exclusion criteria are pregnancy, amedical history of acute bleeding (without cause), venous thrombotic events (VTEs), epilepsy, anticoagulant use and iatrogenic bleeding during surgery (aside from staple line). The primary outcome is postoperative haemorrhage requiring intervention within 30 days postoperatively. Secondary outcome measures are staple line reinforcement, blood loss, duration of surgery, postoperative haemoglobin, vital parameters, minor and major complications, side effects of TXA (nausea, hypotension and VTE), length of hospital stay and directly made costs. ETHICS AND DISSEMINATION: Written informed consent will be obtained from all participants. The protocol has been approved by the Medical Research Ethics Committees United, Nieuwegein, on 7 February 2023 (registration number: R22.102). Results will be disseminated through peer-reviewed publications and conferences. TRIAL REGISTRATION NUMBER: NCT05464394.

A survey of medication and raw food use among canine blood donors.

BACKGROUND: Blood donors are screened for medication use to determine their health status and to ensure that the collection will be safe and efficacious for transfusion. Although stringent medication deferral guidelines exist for human blood donors, no consensus exists as to which medications should be permitted among canine donors. METHODS: A brief survey regarding canine donor screening methods was distributed to an online hematology and transfusion medicine group and included questions pertaining to commonly prescribed medications and consumption of a raw food diet. KEY FINDINGS: The survey results demonstrate that more than half of the respondents accept canine donors given thyroid supplements, whereas respondents were split as to whether they accept canine donors given antihistamines chronically. Most survey respondents exclude canine donors taking anti-inflammatory or anti-itch medications unless in acute circumstances and only after a washout period. More than half of the survey respondents exclude dogs fed a raw food diet. SIGNIFICANCE: The survey results demonstrate that there is no obvious agreement regarding which medications to permit in canine donors. Evidence-based guidelines are needed to inform best practices and the subsequent decisions made by donor programs.

Is drug interference still an issue for pretransfusion testing of patients on anti CD38 and other monoclonal antibody therapies?

Certain therapies that target CD markers on some blood cells can affect pretransfusion testing. Key examples are anti-CD38, CD47 monoclonal antibody (mAb) therapies such as daratumumab (DARA) and magrolimab, which have presented a challenge for transfusion medicine laboratories around the globe. Scientists have been faced with not only introducing a protocol to provide safe blood to patients but also investigating the most effective method to remove the pretransfusion pan-agglutinating interference caused. A number of papers in the last 5 years have reported on various methods to remove pretransfusion interference; however, most of these studies have been conducted only in a few countries. Most recent reviews on this topic have focused on techniques and reagents to remove pretransfusion interference, and dithiothreitol is currently the gold standard for removing DARA interference. However, it was clear from this review that while many laboratories have developed processes for addressing interference in pretransfusion testing, and DARA interference may not be a major issue, there are still laboratories around the world, that may not have adequately addressed this issue. In addition, the impact of mAb interference on widely used techniques such as flow cytometry is unclear.

Characterising the challenges of managing difficult red blood cell alloantibodies in liver transplant recipients.

AIMS: Formation of red blood cell alloantibodies (RBCAs) complicates transfusion support in liver transplantation (LT). Difficult RBCAs (DAs, >3 antibodies or antibodies for which <25% donors are antigen negative) further challenge care. This study characterises DA outcomes relative to non-difficult RBCAs (NDAs). METHODS: Single-centre, retrospective analysis of LT patients (2002-2021). RBCAs were defined as clinically significant antibodies. DAs were compared with NDAs. RESULTS: 89 patients had clinically significant RBCAs (DA=50, NDA=39). More DAs were anti-Jka, anti-M; fewer were anti-E, anti-K (all p<0.05). DA patients often had multiple antibodies (44% vs 12.8% NDA, p=0.0022). Probability of finding antigen-negative blood was lower for DAs (17.4% vs 68.1% NDA, p<0.0001) as was RBCs received (9.4 vs 14.7 units in NDA, p=0.0036). Although survival was similar, patients with DAs had more adverse reactions (8% vs 0%, p=0.128). Some antibodies appeared to occur with specific liver diseases (such as primary sclerosing cholangitis, alcoholic steatohepatitis and recurrent disease); however, due to low sample size, definitive conclusions cannot be made. CONCLUSIONS: DA LT recipients contain >1 RBCA, have a lower probability of finding antigen negative blood and may experience more adverse transfusion event (ATE). Despite this, the incidence of ATEs was still quite low.

Use of flow cytometry method to detect contaminations of platelet suspensions.

In this study, it was aimed to investigate bacterial contamination in apheresis platelet suspensions (APS) by automated blood culture system and flow cytometry method (FCM).33 spiked APS each using 11 bacterial strains (5 standard strains, 6 clinical isolates), were prepared in three different dilutions (1-10, 10-50, 50-100 cfu/mL), incubated in two different temperatures (35-37 °C and 22-24 °C) and different incubation times (18-96 h) evaluated by FCM. This three different dilutions were also inoculated into special platelet culture bottles (BacT/ALERT® BPA) and loaded into the blood culture system. Additionally 80 APSs routinely prepared in the Transfusion Center were evaluated by both FCM and the blood culture system. Platelets were lysed by freeze-thaw method.All spiked samples were positive with BacT/ALERT® BPA in 12-18 h. In 96 h incubation at 22-24 °C, the presence of bacteria was detected by FCM in all other samples (31/33) except low dilutions (1-10 and 10-100 CFU/ml) of K.pneumoniae standard strain. In the 35-37 °C, the presence of bacteria was detected by FCM in all samples (33/33) after 48 h of incubation. In routine APS one sample detected as positive (Bacillus simplex) with BacT/ALERT® BPA and no positivity was detected by FCM.The freeze-thaw method, which we have optimized for the lysis of platelets, is very practical and can be easily applied. The BacT/ALERT® system has been found to be very sensitive in detecting bacterial contamination in PSs. Flow cytometry method has been found to be successful, fast, easy to use and low cost in detecting bacterial contamination in PSs.

Increase of Phosphoprotein Expressions in Amotosalen/UVA-Treated Platelet Concentrates.

Background: Pathogen inactivation treatment (PIT) has been shown to alter platelet function, phenotype, morphology and to induce a faster aging of platelet concentrates (PCs). Key pieces of information are still missing to understand the impacts of PITs at the cellular level. Objectives: This study investigated the impact of amotosalen/UVA on PCs, from a post-translational modifications (PTM) point of view. Phosphoproteomic analyses were conducted on resting platelets, right after the amotosalen/UVA treatment and compared with untreated PCs. Method: A two-arm study setting was carried out to compare PIT (amotosalen/UVA) to untreated PCs, on day 1 post-donation. Based on a pool-and-split approach, 12 PCs were split into two groups (treated and untreated). Quantitative phosphoproteomics was performed using TMT technology to study the changes of phosphoproteins right after the PIT. Results: A total of 3,906 proteins and 7,334 phosphosites were identified, and 2,473 proteins and 2,214 phosphosites were observed in at least 5 to 6 replicates. Compared to untreated platelets, PIT platelets exhibited an upregulation of the phosphorylation effects, with 109 phosphosites identified with a higher than 2-fold change. Two pathways were clearly identified. The mitogen activated protein kinases (MAPKs) cascade, which triggers the granule secretion and the activation of the pS15 HSPB1. One of the shape change pathways was also observed with the inhibition of the Threonine 18 and Serine 19 phosphorylations on myosin light chain (MLC) protein after the amotosalen/UVA treatment. Conclusions: This work provides a deep insight into the impact of amotosalen/UVA treatment from a phosphoprotein viewpoint on resting platelets. Clear changes in phosphorylation of proteins belonging to different platelet pathways were quantified. This discovery corroborates previous findings and fills missing parts of the effect of photochemical treatments on platelets.

When you think you should transfuse don't!

A 48-year-old female presented to the emergency department with severe fatigue. Admission laboratory test results were hemoglobin 6.6 g/dL, platelet count 287,000/µL, and white blood cell count 25,200/µL. Lactate dehydrogenase was elevated at 898 U/L, haptoglobin was markedly decreased (< 31 mg/dL), indirect bilirubin was elevated (5.3 mg/dL), and the absolute reticulocyte count was low at 0.0050/µL. A sample was sent to the immunohematology reference laboratory. The direct antiglobulin test immunoglobulin G was negative; C3 was 1+. All cells were reactive at immediate spin phase, indirect antiglobulin testing (IAT) with polyethylene glycol, with low ionic strength saline, neat, prewarm, and in the solid phase. All cells were nonreactive at IAT-ficin. Additional testing included a cold antibody titer that was 1:4096 and thermal amplitude studies demonstrating reactivity of 2+ at 37°C. These results were consistent with a clinically significant anti-Pr and cold agglutinin disease (CAD). Although rituximab is effective in autoimmune hemolytic anemia, this may take weeks. The patient was treated with pegcetacoplan, a pegylated peptide that targets C3 inhibiting hemolysis. The patient was discharged on day 29 with a hemoglobin of 8 g/dL. This is a report of one of the first patients successfully treated with pegcetacoplan for CAD.

The effect of serious game and problem-based learning on nursing students' knowledge and clinical decision-making skill regarding the application of transfusion medicine in pediatric nursing.

PURPOSE: Comparing the effect of serious game and problem-based learning on nursing students' knowledge and clinical decision-making skill regarding the application of transfusion medicine in pediatric nursing. DESIGN AND METHODS: In this quasi-experimental study, 76 undergraduate nursing students were enrolled through a convenience sampling method, and were allocated to one of the three groups of serious game, problem-based learning, and control through the block randomization method. Data were collected using a valid and reliable 3-part researcher-made tool, completed before and two weeks after the intervention. Statistical analysis was performed using paired t-test, analysis of covariance, and Bonferroni post hoc test. A significance level of <0.05 was considered. RESULTS: After the intervention, mean scores of both knowledge and clinical decision-making skill increased significantly in both intervention groups (p < 0.05). Mean post-test scores of both knowledge and clinical decision-making skill in the serious game group, and only clinical decision-making skill in the problem-based learning group were significantly higher than the control group (p < 0.05). However, no significant difference was observed regarding mean post-test scores of both knowledge and clinical decision-making skill between the intervention groups (p > 0.05). CONCLUSIONS: Both serious game and problem-based learning are proven to be effective in improving nursing students' knowledge and clinical decision-making skill regarding the application of transfusion medicine in pediatric nursing. PRACTICE IMPLICATIONS: Since learning now occurs beyond classrooms and the new generation of students spend most of their time in virtual places, utilizing technology-based teaching methods like serious games can benefit both educators and students by providing continuous education, saving their time and expenses, etc.

Transfusion Camp Rwanda 2023: A train-the-trainer workshop establishing locally driven leadership in knowledge translation and sustainability in transfusion medicine education.

BACKGROUND AND OBJECTIVES: Blood transfusion is performed daily in hospitals. Gaps exist between transfusion guidelines and day-to-day clinical care. These gaps are prevalent in resource-limited settings due to scarce continuing medical education. Transfusion Camp Rwanda aims to bridge this gap by (1) delivering context-appropriate up-to-date education, (2) teaching participants how to independently deliver a case-based curriculum and (3) identifying strategies to promote change in transfusion practice in Rwanda. MATERIALS AND METHODS: In May 2023, a multidisciplinary team from Canada and Rwanda carried out a Transfusion Camp train-the-trainer workshop for clinicians from all five provinces in Rwanda. Participants attended in-person lectures, seminars and workshop group discussions on the implementation of the Rwanda National Directives on Rational Use of Blood and Blood Components. Course feedback was based on the Kirkpatrick Model of Training and Evaluation. RESULTS: Fifty-one physicians and laboratory technicians participated in the course. Confidence in caring for patients based on transfusion guidelines was self-rated as 'excellent' by 23% of participants before and 77% after, while 84% reported they planned to teach Transfusion Camp to others and 100% responded that they will apply course content to clinical practice. Workshop groups recommended strategies to improve transfusion medicine practice in Rwanda in four domains: Communication, Institutional Approval, Practice Audits and Education. CONCLUSION: Transfusion medicine education in Rwanda using a train-the-trainer approach was well-received by participants and allowed for a more detailed understanding of the local medical and educational environment. These observations can inform the further expansion of the Transfusion Camp Rwanda project.

Self-management needs, strategies and support for sickle cell disease in developing countries: a scoping review protocol.

INTRODUCTION: Sickle cell disease (SCD) poses a significant global health burden, particularly affecting individuals in developing countries with constrained healthcare resources. While research on self-management in the context of SCD is emerging, it has predominantly focused on primary studies, and there is a notable dearth of evidence synthesis on SCD self-management in developing countries. This scoping review aims to identify and map self-management needs of individuals living with SCD, the strategies they employed to meet those needs, and the support systems available to them. METHODS AND ANALYSIS: The review will be conducted following the Arksey and O'Malley's (2005) 29 framework to comprehensively examine the landscape of SCD self-management research. Searches will be performed in PubMed, Scopus, Embase and Dimensions AI, with additional searches in other databases and grey literature. Indexed literature published in English from inception to January 2024 will be included. Reference list from included studies will also be searched manually. Two teams will be constituted to independently screen titles, abstracts and full text against the eligible criteria. Data will be extracted from included studies onto a customised data extraction form. ETHICS AND DISSEMINATION: Ethical approval is not required for this review due to the fact that it synthesises information from available publications. The findings will be disseminated through publication in a peer-reviewed journal. Also, the findings will possibly be presented at relevant international and national conferences. This protocol has already been registered with the Open Science Framework. The study characteristics such as design and setting will be descriptively analysed and presented as graphs, tables and figures. Thematic analysis will also be conducted based on the study objectives and presented as a narrative summary.