Thalassemia and malignancies: Updates from the literature.

Thalassemia management has undergone significant development with the advancement in iron chelation therapy, which has led to a prolonged life expectancy. This has been accompanied by the emergence of several new morbidities and chronic diseases, including cancer. Over the years, multiple cases of solid and hematologic malignancies in thalassemia patients have been reported in the literature, with no clear mechanism for the development of cancer in these patients despite a number of potential mechanisms. However, the results of many studies have been contradictory regarding the risk of development of malignancies in thalassemia. The present review aims to discuss the available data on cancer and thalassemia in the literature, with the latest updates regarding possible malignancy development mechanisms, risks, and the most commonly reported types.

Standardized Calcium Replacement in Massive Transfusion: A Quality Improvement Project.

BACKGROUND: Hypocalcemia is common in trauma patients receiving massive transfusion protocol and often leads to worsening coagulopathies. Despite the identified problem and recommendations for replacement, few institutions have implemented a standardized calcium replacement protocol. OBJECTIVE: This study aims to assess whether a revised massive transfusion protocol, including standardized calcium replacement, increases the incidence of calcium administration in trauma patients receiving massive transfusion protocol. METHODS: This quality improvement project used a retrospective pre-/postdesign to study the revision of the current facility's massive transfusion protocol to include calcium replacement and ionized calcium monitoring at an urban Level I academic trauma center. Pre- and postintervention data were collected from January 2022 through October 2022 to determine the number of times massive transfusion protocol was ordered, ionized calcium monitoring, and calcium administration rates. Feedback regarding the protocol was collected throughout the monitoring period and was utilized in the final analysis. RESULTS: A total of 40 patients received massive transfusion protocol, preintervention, 18 of 23 (78%) received calcium supplementation, postintervention, 15 of 16 (98%) were treated. The majority of protocol activations occurred in the trauma bay (79%) and postintervention; ionized calcium monitoring dropped by 14%. CONCLUSION: This study found that the addition of standardized calcium replacement improved administration rates of calcium in this patient population. Ongoing research will ensure the recommended changes improve the identified shortcomings and that patients maintain adequate ionized calcium levels with the current dosing parameters.

High Cell Saver Autotransfusion is Associated With Perioperative Medical Complications in Adult Spinal Deformity Patients.

STUDY DESIGN: A retrospective, propensity-matched observational study. OBJECTIVE: To assess the impact of cell saver (CS) homologous transfusion on perioperative medical complications in adult patients undergoing spinal deformity surgery. SUMMARY OF BACKGROUND DATA: Despite many endorsing its use, many analyses still refute the efficacy of CS on decreasing total perioperative allogenic red blood cell transfusions, cost efficiency, and its effect on perioperative complications. METHODS: Adult patients who underwent spinal deformity surgery at a single center between 2015 and 2021 were retrospectively reviewed. Patient-specific, operative, radiographic, and 30-day complications/readmission data were collected for further analysis. Two methods were utilized to test our hypothesis: (1) absolute threshold model: two cohorts created among patients who received ≥550 mL of CS intraoperatively and those who received less; (2) adjusted ratio model: two cohorts created dependent on the ratio of CS to estimated blood loss (EBL). Propensity-score matching and various statistical tests were utilized to test the association between CS and perioperative medical complications. RESULTS: Two hundred seventy-eight patients were included in this analysis with a mean age of 61.3±15.7yrs and 67.6% being female. Using the first method, 73 patients received ≥550 mL of CS, and 205 received less. Propensity-score matching resulted in 28 pairs of patients. 39.3% of patients with ≥550 mL CS required readmission within 30 days compared with 3.57% of patients in the <550 mL cohort ( P =0.016), despite a nearly identical proportion of patients requiring intraoperative blood transfusions ( P >0.9999). Using the second method, 155 patients had CS/EBL<0.33 and 123 with CS/EBL ≥0.33. 5.16% and 21.9% among patients with CS/EBL<0.33 and CS/EBL≥0.33, respectively, were readmitted by the 30-day marker ( P <0.0001). CONCLUSIONS: Our findings indicate that greater CS volumes transfused are associated with higher rates of 30-day readmissions. Thus, surgeons should consider limiting CS volume intraoperatively to 550 mL and when greater volumes are required or preferred, ensuring that the ratio of CS:EBL remains under 0.33.

Autologous versus allogeneic blood transfusion: A comparative study of the peri-operative outcomes in a tertiary care hospital in South India amidst the COVID-19 pandemic.

Autologous blood transfusion is known to have advantages over allogeneic transfusion. The COVID-19 pandemic intensified the already existing shortage of allogeneic blood supply. We carried out a study during this period with the aim to elicit the effects of autologous blood collection and transfusion, to compare the peri-operative outcomes of autologous and allogeneic transfusion practices and also to assess the influence of the autologous transfusion programme in the Blood Centre inventory. It was prospective observational comparative study among neurosurgical and vascular surgical patients in a tertiary care centre in South India. 141 patients were allocated into Group I (n = 71) who received autologous transfusion and those who received conventional allogeneic transfusion were clustered as Group II (n = 72) for analysis. We employed Acute Normovolemic Hemodilution (ANH), Pre-deposit Autologous Donation (PAD) and Intra-operative Cell Salvage (ICS) as various modalities for autologous blood collection. In our study, 43 (60.6%) from Group I received exclusive autologous blood transfusion, whereas 28 (39.4%) required additional allogeneic transfusion. No significant difference in hemoglobin, hematocrit, platelet count and INR were observed between the groups post transfusion. Significant difference was observed in the thoracoabdominal aortic aneurysm (TAAA) patients with respect to duration of ICU stay (2.7 ± 1.1 days in Group I and 6.2 ± 0.8 days in Group II; p = 0.002) and re-exploration due to bleeding (16.7% in Group I and 40% in Group II; p = 0.048). Autologous blood transfusion is safe and effective. It can be employed as routine practice and also during any acute shortage or pandemic.

Cost-Effectiveness of Cell Salvage in Trauma Blood Transfusions.

INTRODUCTION: Despite the increasing amount of evidence supporting its use, cell salvage (CS) remains an underutilized resource in operative trauma care in many hospitals. We aim to evaluate the utilization of CS in adult trauma patients and associated outcomes to provide evidence-based recommendations. METHODS: A systematic review was conducted using PubMed, Google Scholar, and CINAHL. Articles evaluating clinical outcomes and the cost-effectiveness of trauma patients utilizing CS were included. The primary study outcome was mortality rates. The secondary outcomes included complication rates (sepsis and infection) and ICU-LOS. The tertiary outcome was the cost-effectiveness of CS. RESULTS: This systematic review included 9 studies that accounted for a total of 1119 patients that received both CS and allogeneic transfusion (n = 519), vs allogeneic blood transfusions only (n = 601). In-hospital mortality rates ranged from 13% to 67% in patients where CS was used vs 6%-65% in those receiving allogeneic transfusions only; however, these findings were not significantly different (P = .21-.56). Similarly, no significant differences were found between sepsis and infection rates or ICU-LOS in those patients where CS usage was compared to allogeneic transfusions alone. Of the 4 studies that provided comparisons on cost, 3 found the use of CS to be significantly more cost-effective. CONCLUSIONS: Cell salvage can be used as an effective method of blood transfusion for trauma patients without compromising patient outcomes, in addition to its possible cost advantages. Future studies are needed to further investigate the long-term effects of cell salvage utilization in trauma patients.

Hypocalcemia in Trauma is Determined by the Number of Units Transfused, Not Whole Blood Versus Component Therapy.

INTRODUCTION: Blood component resuscitation is associated with hypocalcemia (HC) (iCal <0.9 mmol/L) that contributes to coagulopathy and death in trauma patients. It is unknown whether or not whole blood (WB) resuscitation helps mitigate the risk of HC in trauma patients. We hypothesized that calcium homeostasis is maintained and mortality improved in patients who only receive WB. MATERIALS AND METHODS: This is a retrospective review of all adult trauma patients who received WB from July 2018 to December 2020. Variables included transfusions, ionized calcium levels, and calcium replacement. Patients were characterized as follows based on blood products received: WB or WB with other blood components. Groups were compared with respect to HC, correction of HC, 24 h, and inpatient mortality. RESULTS: Two hundred twenty-three patients received WB and met the inclusion criteria. 107 (48%) received WB only. HC occurred in 13% of patients who received more than one WB unit compared to 29% of WB and other blood component patients (P = 0.02). WB patients received less calcium replacement (median 250 mg versus 2000 mg, P < 0.01). HC and total units transfused within 4 h were associated with mortality in the adjusted model. HC significantly increased after 5 units of blood products were transfused, regardless of product type. WB was not protective against HC. CONCLUSIONS: HC and failure to correct HC are significant risk factors for mortality in trauma. Resuscitations with WB only and WB in combination with other blood components are associated with HC especially when more than 5 units of any blood product are transfused. Calcium supplementation should be prioritized in any large volume transfusion, regardless of blood product type.

Determinantes externos de la disponibilidad de sangre / External Determinants of Blood Availability

El acceso universal a las transfusiones de sangre depende de diversos factores localizados dentro y fuera de los servicios de salud. Motivados por ser este un tema infrecuente, el trabajo tiene el objetivo de analizar desde una perspectiva multidimensional y holística los determinantes externos de la disponibilidad de sangre para las transfusiones. El desarrollo socioeconómico constituye un importante factor dinamizador porque condiciona, de cierta manera, la infraestructura del sistema sanitario e incide sobre los factores epidemiológicos, sociodemográficos y jurídicos. A su vez, estos influyen en la organización de las actividades y la complejidad de la asistencia sanitaria, factores que otorgan un sentido positivo o negativo a la disponibilidad de sangre. Tomarlos en consideración al diseñar las políticas de salud relacionadas con el uso de la sangre permite una mayor precisión en las intervenciones locales que contribuyan a mejorar la seguridad de las transfusiones(AU)

Universal access to blood transfusions depends on a number of localized factors both inside and outside health services. Motivated by being an infrequent topic, this work aims to analyze from a multidimensional and holistic perspective the external determinants of the availability of blood for transfusions. Socio-economic development is an important dynamic factor because it conditions, in a certain way, the infrastructure of the health system and affects epidemiological, sociodemographic and legal factors. In turn, these influence the organization of activities and the complexity of health care, factors that give a positive or negative meaning to the availability of blood. Taking them into consideration when designing health policies related to the use of blood allows greater precision in local interventions that contribute to improving the safety of transfusions(AU)

Is a Patient Blood Management programme economically reasonable?

PURPOSE OF REVIEW: The value of healthcare is defined as the achieved health outcome in relation to the incurred costs. Patient Blood Management (PBM) is a multidisciplinary, evidence-based and patient-centred concept to optimize the patients' red blood cell mass, minimize blood loss and bleeding and to secure the physiological reserve, including the promotion of evidence-based transfusion strategies. This review describes the healthcare value and the cost effectiveness of single PBM measures as well as the implementation of comprehensive PBM programmes. RECENT FINDINGS: Overall, measures improving surgical outcome and reducing hospital length of stay, such as intravenous iron supplementation in iron deficient anaemic patients, the use of antifibrinolytic agents for the treatment of bleeding, the use of cell salvage and adherence to an evidence-based transfusion strategy, are associated with cost savings. SUMMARY: Although several single PBM measures have been shown to be effective and cost-efficient, it remains challenging to compare the results among differing healthcare systems.

Health state utilities for beta-thalassemia: a time trade-off study.

BACKGROUND: Beta-thalassemia (BT) is an inherited blood disorder characterized by reduced levels of functional hemoglobin resulting in phenotypes ranging from clinically asymptomatic to severely anemic. Patients with BT may require lifelong regular blood transfusions supported by appropriate iron chelation therapy (ICT). This study aimed to determine how the UK general population values BT health states associated with differing transfusion burden and ICT. METHODS: Composite time trade-off (cTTO) methodology was employed to elicit health state utilities in BT. Relevant BT literature related to symptom and quality-of-life impact, including physical, functional, and emotional well-being, and safety profiles of BT treatments were considered when drafting health state descriptions. Eleven health state descriptions were developed and validated by hematologists and patient advocates for clinical accuracy and completeness. 200 individuals from the UK general population participated in the cTTO interviews. RESULTS: The mean age of participants was 41.50 years (SD 16.01, range 18-81); 88 (46.8%) were female. Utility values ranged from 0.78 (SD 0.34) for non-transfusion dependent BT with oral ICT to 0.37 (SD 0.50) for high transfusion burden with subcutaneous ICT in transfusion-dependent BT. CONCLUSIONS: This study provides health utilities for a range of BT health states from the UK general population perspective. Importantly, lower transfusion burden and lower burden of anemia were associated with higher utilities. To a lesser extent, differential modes of ICT were found to impact utility valuations in patients with BT. The utilities obtained in this study can be employed as inputs in cost-effectiveness analyses of BT therapies.

Patient Blood Management programs for post-partum hemorrhage.

Patient blood management (PBM) strategies aim to maintain hemoglobin concentration, optimize hemostasis, and minimize blood loss to improve patient outcomes. Because postpartum hemorrhage (PPH) is a leading cause of maternal mortality and blood product utilization, PBM principles can be applied in its therapeutic approach. First, pre-operative identification of risk factors for PPH and identification of peri-delivery anemia should be conducted. Iron supplementation should be used to optimize hemoglobin concentration before delivery; it can also be used to treat anemia in the postpartum period after severe PPH. Both acute normovolemic hemodilution and intraoperative cell salvage can be effective techniques to reduce allogeneic blood transfusion during or after surgical procedures. Furthermore, these strategies appear to be safe when used in the pregnant population.

Patient blood management when blood is not an option: a report of two cases.

There are many patients who refuse to receive blood transfusions for religious or other reasons. Bloodless medicine and surgery are no longer new concept, but patients who refuse blood transfusion are still transferred to the bloodless center, regardless of patients' intention, for treatment. Here, we discuss the need for patient blood management when blood is not an option to treat them. Two patients of advanced age were transferred to our bloodless center due to refusal of transfusion. They are Jehovah's Witnesses and refused to receive blood transfusion despite life-threatening anemia and severe underlying diseases. Patient blood management protocols including iron supplementation, subcutaneous erythropoietin, folic acid and vitamin B were implemented to improve hematopoiesis, and supportive care was also performed to treat underlying diseases. Levels of Hemoglobin/Hematocrit and their symptom gradually improved about a week after treatment, and their condition had gradually stabilized. They were discharged safely. We treated patients of advanced age with severe underlying diseases and life-threatening anemia using patient blood management due to refusal of a blood transfusion. The patient blood management may be a useful alternative strategy, which meet the needs of patients who refuse blood transfusions as well as the need to reduce the use of blood products due to limited supply.

Fatigue in post-acute sequelae of SARS-CoV2 (PASC) treated with oxygen-ozone autohemotherapy - preliminary results on 100 patients.

OBJECTIVE: Post-acute sequelae of SARS-CoV2 infection (PASC) are a novel terminology used to describe post-COVID persistent symptoms, mimicking somehow the previously described chronic fatigue syndrome (CFS). In this manuscript, we evaluated a therapeutical approach to address PASC-derived fatigue in a cohort of past-COVID-19 positive patients. PATIENTS AND METHODS: A number of 100 patients, previously diagnosed as COVID-19 positive subjects and meeting our eligibility criteria, was diagnosed having PASC-related fatigue. They were recruited in the study and treated with oxygen-ozone autohemotherapy (O2-O3-AHT), according to the SIOOT protocol. Patients' response to O2-O3-AHT and changes in fatigue were measured with the 7-scoring Fatigue Severity Scale (FSS), according to previously published protocols. RESULTS: Statistics assessed that the effects of O2-O3-AHT on fatigue reduced PASC symptoms by 67%, as a mean, in all the investigated cohort of patients (H = 148.4786 p < 0.0001) (Figure 1). Patients following O2-O3-AHT therapy, quite completely recovered for PASC-associated fatigue, a quote amounting to about two fifths (around 40%) of the whole cohort undergoing ozone treatment and despite most of patients were female subjects, the effect was not influenced by sex distribution (H = 0.7353, p = 0.39117). CONCLUSIONS: Ozone therapy is able to recover normal functionality and to relief pain and discomfort in the form of PASC-associated fatigue in at least 67% of patients suffering from post-COVID sequelae, aside from sex and age distribution.

Massive transfusions and severe hypocalcemia: An opportunity for monitoring and supplementation guidelines.

BACKGROUND: Massive transfusion protocols (MTPs) are associated with severe hypocalcemia, contributing to coagulopathy and mortality in severely injured patients. Severity of hypocalcemia following massive transfusion activation and appropriate treatment strategies remain undefined. STUDY DESIGN AND METHODS: This was a retrospective study of all MTP activations in adult trauma patients at a Level 1 trauma center between August 2016 and September 2017. Units of blood products transfused, ionized calcium levels, and amount of calcium supplementation administered were recorded. Primary outcomes were ionized calcium levels and the incidence of severe ionized hypocalcemia (iCa ≤1.0 mmol/L) in relation to the volume of blood products transfused. RESULTS: Seventy-one patients had an MTP activated during the study period. The median amount of packed red blood cells (PRBCs) transfused was 10 units (range 1-52). A total of 42 (59.1%) patients had periods of severe hypocalcemia. Patients receiving 13 or more units of PRBC had a greater prevalence of hypocalcemia with 83.3% having at least one measured ionized calcium ≤1.0 mmoL/L (p = .001). The number of ionized calcium levels checked and the amount of supplemental calcium given in patients who experienced hypocalcemia varied considerably. DISCUSSION: Severe hypocalcemia commonly occurs during MTP activations and correlates with the number of packed red blood cells transfused. Monitoring of ionized calcium and amount of calcium supplementation administered is widely variable. Standardized protocols for recognition and management of severe hypocalcemia during massive transfusions may improve outcomes.

Pathogen reduction of whole blood: Supplementing fibrinogen partly corrects clot formation in a massive transfusion model.

BACKGROUND: The use of whole blood (WB) to treat trauma patients is becoming more common. Similar to the treatment of individual components, pathogen inactivation (PI) technologies are available to treat WB. The impact of PI on WB function is not well understood. This study investigated the impact of PI of WB with riboflavin/ultraviolet (UV) light on its hemostatic function by modeling transfusion scenarios for trauma patients and assessing transfusion efficacy by rotational thromboelastometry (ROTEM). As fibrinogen is affected by PI of WB, the effect of fibrinogen supplementation commonly used in trauma patients was also analyzed in this model. STUDY DESIGN AND METHODS: Trauma transfusion scenarios were simulated by mixing untreated WB or WB treated with the Mirasol PI technology (riboflavin/UV) in different ratios with hemodiluted blood, and the thromboelasticity was monitored by ROTEM. The impact of supplementation with the fibrinogen concentrate RiaSTAP was investigated in this model. RESULTS: Pathogen-inactivated WB (PI-WB) showed decreased activity in the hemostatic profile compared to the untreated control. Hemodiluted blood at a hematocrit (hct) of 20%, which was reconstituted with PI-WB or untreated WB, exhibited increased alpha values, maximum clot firmness, and clot formation time. Simulating transfusion scenarios by blood replacement with PI-WB resulted in a significant difference in ROTEM parameters between reconstituted PI-treated and -untreated WB (p ≥ .05). The effect of PI treatment waned when PI-WB was enriched with fibrinogen. CONCLUSION: ROTEM investigations suggest that PI treatment has a negative impact on WB clot formation unless fibrinogen supplementation is used.

The risk of hyperbilirubinemia in term neonates after placental transfusion - a randomized-blinded controlled trial.

OBJECTIVE: We aimed to demonstrate non-inferiority of delayed cord clamping (DCC) and cord milking (CM) in comparison to early cord clamping (ECC) in the incidence of hyperbilirubinemia requiring phototherapy. MATERIAL AND METHODS: 467 of maternal-foetal dyads were screened for eligibility. 389 term infants, of breastfeeding, non-smoking mothers were randomized to receive ECC ( < 40 s), DCC (1-2 min) or CM (4 times towards the neonate). The primary outcome was defined as hyperbilirubinemia requiring phototherapy. RESULTS: 307 patients were included in the analysis. CM did not increase the risk of phototherapy RR 11.27 95% CI (0.80; 2.04). Similar results were achieved when comparing DCC and ECC, RR 1.29 95% CI (0.82; 2.05). This was also true for CM vs DCC, RR 0.99 95% CI (0.64; 1.52). The prevalence of total serum bilirubin (TSB) at 24-48 hours was 10.8 mg/dL; 10.33 mg/dL and 11.39 in ECC, CM and DCC group respectively. Transcutaneous bilirubin (TcB) levels at 24-48 h were 7.58 mg/dL, 7.89 mg/dL and 7.60 mg/dL in the ECC, CM and DCC respectively. None of the neonates met exchange transfusion criteria or symptomatic polycythaemia. CONCLUSIONS: Our study suggests that placental transfusion is not associated with hyperbilirubinemia requiring phototherapy or exchange transfusion.

Preoperative autologous platelet pheresis reduces allogeneic platelet use and improves the postoperative PaO2/FiO2 ratio in complex aortic surgery: a retrospective analysis.

OBJECTIVES: An autologous platelet-rich plasma pheresis (aPP) strategy can harvest partial whole blood that is separated into erythrocytes, plasma and platelets, and can reduce blood loss and transfusion during cardiovascular surgery using cardiopulmonary bypass (CPB). However, the blood and organ conservation effects of this technique have not been confirmed in the context of complex aortic surgery. METHODS: Perioperative records of 147 adult patients who underwent complex aortic surgery were analysed retrospectively. RESULTS: All patients received regular blood conservation treatment, and 57 patients received aPP. Whether or not the participants were propensity matched, decreased platelet and cryoprecipitate transfusions were found in the aPP group (both P < 0.001), but there were non-significant differences in erythrocyte transfusion, Sequential Organ Failure Assessment scores and other outcomes when compared with the same parameters in the non-aPP group. The aPP group had a higher arterial oxygen partial pressure to inhaled oxygen concentration ratio on postoperative days 1, 2 and 7 than the non-aPP group (P < 0.001, P < 0.001 and P = 0.048, respectively). CONCLUSIONS: The utilization of aPP was associated with a reduction in allogeneic platelet and cryoprecipitate transfusions as well as minor lung-protective effects during complex aortic surgery using CPB.

How to implement of patient blood management pillar 1: An Italian expert opinion based on a "bundles" approach.

Worldwide iron anemia is a common disorder with a significant economic burden on health-care systems. Red blood cell transfusion is the mainstay to correct anemia in surgical settings, but it is also an overused procedure and recent data support its possible role in worsening patient outcomes. Patient Blood Management (PBM) is a multidisciplinary approach to optimize hemostasis, manage anemia, minimize iatrogenic blood loss, and improve tolerance to anemia. The present paper aims to provide a "bundles" approach, based on several preoperative anemia management measures, to implement PBM Pillar 1 in clinical practice.

How I treat gastrointestinal bleeding in congenital and acquired von Willebrand disease.

Gastrointestinal (GI) bleeding is distinctive of severe von Willebrand disease (VWD), generally arising in older patients; in most cases, blood transfusion and hospitalization are required. The presence of arteriovenous malformations is often described when endoscopic examinations are performed. Patients with congenital type 3, 2A, and 2B are those most frequently affected by this symptom, possibly due to the loss of high-molecular-weight multimers of von Willebrand factor (VWF). GI bleeding can also occur in patients affected by acquired von Willebrand syndrome. Endoscopic examination of the GI tract is necessary to exclude ulcers and polyps or cancer as possible causes of GI bleeding. In congenital VWD, prophylaxis with VWF/factor VIII concentrates is generally started after GI-bleeding events, but this therapy is not always successful. Iron supplementation must be prescribed to avoid chronic iron deficiency. Possible rescue therapies (high-dose statins, octreotide, thalidomide, lenalidomide, and tamoxifen) were described in a few case reports and series; however, surgery may be necessary in emergency situations or if medical treatment fails to stop bleeding. In this article, we present several clinical cases that highlight the clinical challenges of these patients and possible strategies for their long-term management.

Beta Thalassemia: New Therapeutic Options Beyond Transfusion and Iron Chelation.

Hemoglobinopathies are among the most common monogenic diseases worldwide. Approximately 1-5% of the global population are carriers for a genetic thalassemia mutation. The thalassemias are characterized by autosomal recessive inherited defects in the production of hemoglobin. They are highly prevalent in the Mediterranean, Middle East, Indian subcontinent, and East and Southeast Asia. Due to recent migrations, however, the thalassemias are now becoming more common in Europe and North America, making this disease a global health concern. Currently available conventional therapies in thalassemia have many challenges and limitations. A better understanding of the pathophysiology of ß-thalassemia in addition to key developments in optimizing transfusion programs and iron-chelation therapy has led to an increase in the life span of thalassemia patients and paved the way for new therapeutic strategies. These can be classified into three categories based on their efforts to address different features of the underlying pathophysiology of ß-thalassemia: correction of the globin chain imbalance, addressing ineffective erythropoiesis, and improving iron overload. In this review, we provide an overview of the novel therapeutic approaches that are currently in development for ß-thalassemia.

Variations in hemoglobin measurement and eligibility criteria across blood donation services are associated with differing low-hemoglobin deferral rates: a BEST Collaborative study.

BACKGROUND: Determination of blood donor hemoglobin (Hb) levels is a pre-requisite to ensure donor safety and blood product quality. We aimed to identify Hb measurement practices across blood donation services and to what extent differences associate with low-Hb deferral rates. METHODS: An online survey was performed among Biomedical Excellence for Safer Transfusion (BEST) Collaborative members, extended with published data. Multivariable negative-binomial regression models were built to estimate adjusted associations of minimum donation intervals, Hb cut-offs (high, ≥13.5 g/dL in men or ≥ 12.5 g/dL in women, vs. lower values), iron monitoring (yes/no), providing or prescribing iron supplementation (yes/no), post-versus pre-donation Hb measurement and geographical location (Asian vs. rest), with low-Hb deferral rates. RESULTS: Data were included from 38 blood services. Low-Hb deferral rates varied from 0.11% to 8.81% among men and 0.84% to 31.85% among women. Services with longer minimum donation intervals had significantly lower deferral rates among both women (rate ratio, RR 0.53, 95%CI 0.33-0.84) and men (RR 0.53, 95%CI 0.31-0.90). In women, iron supplementation was associated with lower Hb deferral rates (RR 0.47, 95%CI 0.23-0.94). Finally, being located in Asia was associated with higher low-Hb deferral rates; RR 9.10 (95%CI 3.89-21.27) for women and 6.76 (95%CI 2.45-18.68) for men. CONCLUSION: Differences in Hb measurement and eligibility criteria, particularly longer donation intervals and iron supplementation in women, are associated with variations in low-Hb deferral rates. These insights could help improve both blood donation service efficiency and donor care.