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Background and Objectives: This report describes the implementation of the first pre‐hospital transfusion service in Latin America, with red blood cell concentrates available for immediate use in mobile land units. Materials and Methods: A partnership was established between the Regional Mobile Emergency Service and the Regional Blood Bank, with further development of protocols for the storage, transportation and transfusion of red blood cell concentrates in an ambulance, with the acquisition of special thermal boxes, a heater for blood components and digital thermometers. Results: The Service was developed from May 2019 and implemented in September 2020, in a mobile intensive care unit serving 11 cities, reaching a population of approximately 480 000 inhabitants. Two red blood cell concentrates, type 'O' RhD negative, are available, stored in a special sealed thermal box and with continuous temperature monitoring. A specific transfusion protocol was developed, in accordance with the institutional protocols of the Hospital and its Blood Bank. Conclusion: This is the first description of a pre‐hospital transfusion service implemented in Latin America, with the potential to reduce the number of deaths from civil trauma and which can be extrapolated to other institutions or countries in similar conditions. [ABSTRACT FROM AUTHOR]

Objectives: Deferasirox is an oral iron chelator with established dose-dependent efficacy for the treatment of iron overload secondary to transfusion. However, there is few data reporting the use of Desferasirox in adult patients with sickle cell disease (SCD) and transfusional iron overload. Methods: We conducted a prospective, single center, nonrandomized study from January 2014 to March 2015 in Campinas, Brazil. Seven patients (five women, median age 50 y.o.) who were followed up on regular transfusion program were treated with a single daily dose of deferasirox (median dose 20 mg/kg). They were monitored for clinical symptoms, renal function and hepatotoxicity. Results: One patient discontinued the study due to lack of compliance. Two patients reported mild to moderate adverse events (gastrointestinal disturbances). Five patients had the drug discontinued due to worsening of renal function. One patient had the drug discontinued due to severe hepatotoxicity that evolved to death; no patient finished the study. Discussion and conclusions: Deferasirox does not appear to be well tolerated in SCD patients older than 40 years, in which complications of the underlying disease are already fully installed. The choice of the ideal iron chelator for this population should include an evaluation of comorbidities and organic dysfunctions, as well as the need to find pharmacogenetic safety markers in this group of patients. [ABSTRACT FROM AUTHOR]

Sickle cell disease (SCD) is a frequent indication for chronic transfusion, which can cause iron overload. Excess iron often affects the liver, but not the heart in SCD. Magnetic resonance (MR) is recommended to detect myocardial iron overload (MIO) but its elevated cost requires optimized indication. We aimed to compile all published data on MIO in SCD upon the description of a fatal case of severe MIO in our institution, and to determine associated risk factors. We performed a systematic review using the PRISMA guidelines in two databases (PubMed and Web of Science). Inclusion criteria were publication in English, patients diagnosed with SCD, and reporting ferritin and MIO by MR. Twenty publications reported on 865 SCD adult and pediatric patients, with at least 10 other cases of MIO. The prevalence of MIO in chronically transfused SCD patients can be estimated to be 3% or less, and is associated with high transfusion burden, top-up transfusions, and low adherence to iron chelation. Cardiac siderosis in SCD is rarely reported, and increased awareness with better use of the available screening tools are necessary. Prospective studies should define the recommended chelation regimens depending on the severity of MIO. • Chronic transfusions can lead to liver and heart iron overload. • Myocardial iron overload is rare, but can be fatal in sickle cell disease. • Transfusion burden and low compliance to chelation are possible risk factors. • Increased awareness and better screening for iron overload are necessary. [ABSTRACT FROM AUTHOR]

Abstract Exosomes may represent an interesting antigenic pulse for new forms of anti-tumor immunotherapy. We evaluated exosomes from serum of patients with acute myeloid leukemia (AML) as an antigenic source for dendritic cells (DC) and the effects upon antitumor cytotoxicity, assessed by the percentage of specific lysis of K562 leukemic cells in co-cultures. Surprisingly, incubation of exosomes with DCs decreased lysis of K562, which may correspond to a mechanism of tumor evasion in vivo. However, when immature DCs were pulsed with exosomes purified from K562 culture supernatants, the lysis of target cells was notably enhanced, associated with a substantial increase in the expression of the maturation marker CD83. Thus, the development of vaccines using patients' exosomes would probably add no benefits to the treatment of AML; alternately, exosomes from cultured cells may represent an effective way for maturing DCs into a cytotoxic phenotype, without the immunosuppression observed with patients' exosomes. [ABSTRACT FROM AUTHOR]

Key Clinical Message This manuscript describes the case of a patient with sickle cell anemia who died of fulminant hepatitis after therapy with the iron chelator Deferasirox. The patient was homozygous for the −1774delG polymorphism in the Abcc2 gene, which raises the concern about the use of hepatotoxic drugs in this specific context. [ABSTRACT FROM AUTHOR]

Objectives: In sickle cell/β-thalassemia, mutations in the corresponding β-globin genes are responsible for complex pathological events resulting in diverse clinical complications. The objective of this study was to provide an overview of the clinical and laboratory characteristics of patients with the syndrome, and of the degree of severity of clinical manifestations resulting from the β-thalassemia mutation. Methods: A retrospective chart review was performed on 46 patients with sickle cell/β-thalassemia (31 Sβo and 15 Sβ+), evaluating hematological parameters and end organ damage. Statistical analyzes were carried out in order to highlight differences between the two groups according to the nature of the thalassemia mutation. Results: As expected, patients with the Sβo phenotype had a higher degree of hematological involvement in comparison to Sβ+ patients; with lower hemoglobin levels, and signs of more intense chronic hemolysis. However, Sβ+ patients were more prone to the occurrence of acute chest syndrome. The impact of the thalassemia mutation upon total body and bone composition was also evident, as Sβo patients presented lower body mass index (BMI) and bone mineral density. The degree of bone damage correlated to lower BMI and hemoglobin levels, as well as plaquetosis, monocytosis and elevated lactate dehydrogenase, possibly reflecting the effects of hemolysis and inflammation upon bone metabolism and body constitution. Conclusions: This study identified significant differences among sickle cell/β-thalassemia patients according to the beta mutation involvement, pointing to an important predictor of disease severity. [ABSTRACT FROM AUTHOR]

Dendritic cells play a fundamental role in the antitumor immunity cycle, and the loss of their antigen-presenting function is a recognized mechanism of tumor evasion. We have recently demonstrated the effect of exosomes extracted from serum of patients with acute myeloid leukemia as important inducers of dendritic cell immunotolerance, and several other works have recently demonstrated the effects of these nanoparticles on immunity to other tumor types as well. The aim of this review was to highlight the recent findings on the effects of tumor exosomes on dendritic cell functions, the mechanisms by which they can lead to tumor evasion, and their manipulation as a possible strategy in cancer treatment. [ABSTRACT FROM AUTHOR]

There is no consensus on the risk-benefit status of preoperative autologous blood donation (PAD) for healthy bone marrow donors and there is concern regarding its impact on the development of pre-surgical anemia. We evaluated the changes in hemoglobin levels related to PAD in 80 bone marrow donors of our institution between 2002 and 2016. Mean Hgb values were compared separately for donors who donated 1 or 2 units, at 3 time-points: before PAD collection, the morning before marrow harvest and soon after harvest. Mean baseline Hgb values did not differ significantly between the 2 groups. After PAD collection, there was a significant drop in Hgb levels for the whole cohort of donors but more pronounced for the group that donated 2 units [1 unit: 12.8(8.9–17.4) × 2 units: 11.55(11.2–12.1), p = 0.045]. However, after marrow harvest, Hgb levels were similar for the 2 groups; 61.2% of all donors required autologous transfusion and none required allogeneic transfusion. Furthermore, baseline Hgb <14.35 g/dL was identified as the sensitive cutoff to predict the need for transfusion after marrow harvest (sensitivity of 52% and specificity of 80.4%, p = 0.001). Thus, our analysis demonstrates the possibility of using hemoglobin thresholds as cutoff points for indication of PAD, tending to a more cost-effective approach. Despite significant declines in Hgb levels after PAD, none of the donors in our cohort required allogeneic transfusion, demonstrating the safety of this procedure. Thus, the indication of PAD remains an option for those who feel insecure despite higher baseline Hgb levels. [ABSTRACT FROM AUTHOR]

Sickle cell disease is a chronic inflammatory condition characterized by elevated levels of inflammatory cytokines, which may be regulated by genetic polymorphisms and could be associated with diverse disease presentations and alloimmunization. The aim of this study was to evaluate Treg and Th17 cell frequencies, cytokine gene polymorphisms, and their association with cytokine expression profile in patients with sickle cell disease. For that purpose, we evaluated the IL intron 3 variable number tandem repeat (VNTR, genotypes 1.1, 1.2, 2.2, and 2.3), IL4-T590C>T, IL6-174G>C, TNFα-308G>A, IL10-819T>C, IL10-592A>C, and IL10-1082A>G polymorphisms and their correlation with TGFβ, IL4, IL6, and IL10 gene expression in sickle cell patients. We observed a significant decrease in Treg frequency together with a substantial increase in Th17 response in patients with sickle cell disease compared with healthy controls ( p < 0.001 and p = 0.014, respectively). There was also a higher prevalence of the IL4-590T/T genotype in patients with sickle cell disease than in Afro-Brazilian descendent controls ( p < 0.001) and higher expression of IL4 in patients with the 1.1 genotype of IL4 intron 3 VNTR ( p = 0.06). Significantly greater gene expression of TGFβ, IL6, and IL10 was observed in sickle cell patients when compared with controls ( p = 0.01, 0.03, and <0.001, respectively). Moreover, higher levels of interleukin-6 and -10 were observed in the group of alloimmunized patients. These new data bring insights into the deregulation in the immune system affecting sickle cell patients and must be further investigated in larger cohorts to better characterize individual variations in immune responses and new markers for disease morbidity. [ABSTRACT FROM AUTHOR]

Background: Brazilian blood banks encourage donors to report postdonation information (PDI) regarding conditions that would lead to deferral in an attempt to retrieve distributed nonconforming blood., Objectives: This study evaluated the profile of donors reporting PDI, the impact on transfusion safety, and the possible impact on the discard of blood products., Subjects and Methods: We analyzed 115 consecutive PDIs between May 2014 and July 2015, a period comprising two dengue-like syndrome (DLS) outbreaks., Results: These PDIs accounted for 87,780 blood donations. The average time for PDIs since donation was 4 (0-23) days and 190 blood components were discarded. DLS accounted for 21.7% of the PDIs analyzed; 11 of the 23 samples tested were nucleic acid test (NAT) positive for dengue and 2 positive for Zika virus (ZIKV). Six of these PDIs were reported after blood components have been transfused: After NAT testing, one of these recipients was diagnosed with dengue and another one with ZIKV infection, both possible transfusions transmitted but without clinical consequences., Conclusion: The high number of recovered blood components due to PDI suggests that PDI remains a great ally in the fight against transfusion-transmitted infections and may be particularly useful during outbreaks of emerging potentially blood-borne pathogens., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Asian Journal of Transfusion Science.)

Objectives: In sickle cell/β-thalassemia, mutations in the corresponding β-globin genes are responsible for complex pathological events resulting in diverse clinical complications. The objective of this study was to provide an overview of the clinical and laboratory characteristics of patients with the syndrome, and of the degree of severity of clinical manifestations resulting from the β-thalassemia mutation., Methods: A retrospective chart review was performed on 46 patients with sickle cell/β-thalassemia (31 Sβ° and 15 Sβ + ), evaluating hematological parameters and end organ damage. Statistical analyzes were carried out in order to highlight differences between the two groups according to the nature of the thalassemia mutation., Results: As expected, patients with the Sβ 0 phenotype had a higher degree of hematological involvement in comparison to Sβ + patients; with lower hemoglobin levels, and signs of more intense chronic hemolysis. However, Sβ + patients were more prone to the occurrence of acute chest syndrome. The impact of the thalassemia mutation upon total body and bone composition was also evident, as Sβ 0 patients presented lower body mass index (BMI) and bone mineral density. The degree of bone damage correlated to lower BMI and hemoglobin levels, as well as plaquetosis, monocytosis and elevated lactate dehydrogenase, possibly reflecting the effects of hemolysis and inflammation upon bone metabolism and body constitution., Conclusions: This study identified significant differences among sickle cell/β-thalassemia patients according to the beta mutation involvement, pointing to an important predictor of disease severity.