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Abstract Introduction A reduced platelet count (PLT) is a frequent post-operative finding in orthopedic surgery patients. Despite its prevalence, the characteristics of post-surgical thrombocytopenia have not been well described. Methods A retrospective chart review was conducted on patients who underwent a knee or hip replacement from 2012 to 2015. Patients who received heparin were excluded. Results A total of 56 patients were analyzed on post-operative days 0 to 4. By day 1, 90.9% of the patients experienced a reduction in their platelet counts. The lowest mean platelet count (nadir) occurred on day 2 (201.3 × 109/L). The average decrease in the platelet count from the baseline was 24% (95%CI: 20.6 - 27.2). The change in the platelet count from the baseline ranged from a 49.6% drop to a 14.2% increase. A substantial portion of patients experienced thrombocytopenia, with 28% occurring on day 2. Platelet counts less than 100 × 109/L occurred only once. The percent decrease in the platelet count from the baseline to any other time point was significantly larger in patients aged > 65 years, compared to patients aged ≤ 65 years (p = 0.007). Specifically, the average drop in the platelet count at the nadir (day 2) relative to the baseline was 27.8% in patients aged > 65 years, compared to 19.5% in patients aged ≤ 65 years. Conclusions A reduction in the platelet count is a frequent post-operative finding in orthopedic surgery patients, even after removing confounding factors, such as heparin exposure, but clinical thrombocytopenia is uncommon. Alternative etiologies should be considered when the platelet count is less than 100 × 109/L. Vigilance should also be considered regarding elderly patients.

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Abstract Introduction Hemorrhagic shock is the main cause of death in the prehospital environment, which highlights the need to standardize measures aiming at bleeding control and volume replacement in this environment. In Brazil, the first prehospital packed red blood cell transfusion service started in September 2020, in Bragança Paulista, state of São Paulo. Objectives Describe the trends and characteristics of patients who received prehospital transfusions prior to hospital treatment during the first year of operation. Methods A retrospective data review was made of all patients who received transfusions from the mobile intensive care unit in Bragança Paulista over one year. Results In this period, 19 patients were transfused. Since activation, the average response time was 20 min. The mean shock indexes before and after blood transfusion were 2.16 and 1.1, respectively. During the course of the 1st year of prehospital transfusions, no blood was wasted and there were no adverse effects. Conclusion Introduction of the prehospital packed red blood cell transfusion service was successful, with significant improvement in hemodynamic parameters.

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Abstract Background Blood component transfusions are a common and often necessary medical practice during the epidemics of dengue. Transfusions are required for patients when they developed severe dengue fever or thrombocytopenia of 10×109/L or less. This study therefore investigated the risk factors, performance and effectiveness of eight different machine-learning algorithms to predict blood component transfusion requirements in confirmed dengue cases admitted to hospital. The objective was to study the risk factors that can help to predict blood component transfusion needs. Methods Eight predictive models were developed based on retrospective data from a private group of hospitals in India. A python package SHAP (SHapley Additive exPlanations) was used to explain the output of the “XGBoost” model. Results Sixteen vital variables were finally selected as having the most significant effects on blood component transfusion prediction. The XGBoost model presented significantly better predictive performance (area under the curve: 0.793; 95 % confidence interval: 0.699-0.795) than the other models. Conclusion Predictive modelling techniques can be utilized to streamline blood component preparation procedures and can help in the triage of high-risk patients and readiness of caregivers to provide blood component transfusions when required. This study demonstrates the potential of multilayer algorithms to reasonably predict any blood component transfusion needs which may help healthcare providers make more informed decisions regarding patient care.

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Abstract Introduction Immune thrombotic thrombocytopenic purpura (iTTP) is characterized by acute systemic microvascular thrombosis and is associated with a high morbidity and mortality, especially in delayed diagnosis (later than 6-7 days from symptoms). iTTP data in Brazil is scarce, so we aimed to characterize the clinical presentation and identify predictors of death risk in patients with this disease in Brazil. Methods In this single-center retrospective study the patients who underwent therapeutic plasma exchange (TPE) for presumptive or confirmed iTTP were evaluated regarding the epidemiological, clinical, laboratorial characteristics and management. Results A total of 50 patients (90 % female), with median age (IQR) of 34.1 (27-47) years, were enrolled, of which 12 (24 %) died. The most frequent symptoms were neurological (96 %), bleeding (76 %), gastrointestinal (52 %), fever (38 %), and cardiovascular (22 %). Neurological focal deficit and cardiovascular symptoms were more frequently observed in the non-survivor group (P = 0.0019 and P = 0.007, respectively). The mean ± SD number of days from beginning of symptoms to first TPE was 12.22 ± 7.91. We identified an association regarding mortality rate with a score MITS ≥ 2 points (P = 0.04), a higher indirect bilirubin (P = 0.0006), a higher number of transfused red blood cell units (P = 0.025), and platelet transfusion (P = 0.027). Conclusion Delayed diagnosis appears to be associated with a higher frequency of neurological symptoms and mortality. Intensity of hemolysis and signs of organ ischemia, such as cardiovascular symptoms and focal neurological deficit, are indicators of death risk.

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Abstract Introduction The COVID-19 pandemic had an important impact on blood bank services. The onset of the pandemic led to a decrease in the number of blood donors. A remote interview would avoid deferred donors from having to travel to the blood bank. We evaluate the feasibility of using telemedicine as an alternative to a face-to-face interview as a first blood donor screening. Methods Our retrospective study included 404 whole blood and platelets donors, who underwent the clinical interview remotely via telemedicine. The deferred donor would not need to go to the blood bank and eligible candidates were required to donate within 7 days. On the day of donation, a mini-interview was held to ensure donor and blood safety. Results The appointments were made from June 2020 to June 2022, including 263 candidates for whole blood (WB) and 141 for platelets (PLTs). At the end of the telemedicine interview, 285 (70.6 %) candidates were considered eligible. Telemedicine was not performed for 60 (14.8 %) candidates due to technical problems (with audio or video) or absences. The deferral rate among candidates who underwent telemedicine pre-screening was 14.6 % and, among eligible donors after telemedicine, only 7 (2.9 %) were unable to donate blood. Conclusion Telemedicine is a viable alternative and a welcome convenience for potential donors to avoid unnecessary travel.

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Abstract High-dose chemotherapy with autologous hematopoietic stem cell transplantation (auto-HSCT) improved 5-year overall survival rates in relapsed/refractory germ cell tumors (GCTs) from 10% to 52%. Nearly 30% of GCT patients are deemed poor mobilizers after receiving several lines of prior therapy. There is limited data available regarding upfront plerixafor use in GCT patients. We predicted upfront plerixafor use would increase the amount of stem cells collected preventing subsequent mobilizations and improve time to curative therapy. A retrospective, single center, chart review of adult GCT patients who received plerixafor upfront for mobilization at a single center between January 1, 2013 and August 31, 2021 was performed. The primary objective was to evaluate the rate of successful peripheral blood CD34+ cell collections. Secondary objectives consisted of describing the impact of plerixafor use on mobilization and assessing auto-HSCT related outcomes. Sixteen patients received plerixafor upfront after an average of three prior lines of therapy (range: 2-5 lines). Successful collection (≥4 × 106 CD34+ cells/Kg collected within four days) was achieved in 15 (94%) patients in a median of one apheresis day (interquartile range: 1-2 days). All patients proceeded to an initial auto-HSCT and 12 patients (75%) completed both transplants as planned. Survival at 12 months was 50%. The significantly higher amount of CD34+ cells collected over less apheresis days demonstrated the clinical utility of upfront plerixafor and its potential to facilitate more efficient stem cell mobilization. There is a need for larger randomized studies with upfront plerixafor use in this unique patient population.

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Abstract Introduction The prime responsibility of blood transfusion services in India is to provide safe blood. The donated blood is tested for human immunodeficiency virus (HIV), hepatitis B virus (HBV), hepatitis C virus (HCV), malaria and syphilis. In India, the screening of donated blood for syphilis is performed by rapid plasma reagin (RPR) or venereal disease research laboratory (VDRL), whereas the World Health Organization (WHO) recommends screening of syphilis in blood donors by enzyme-linked immunosorbent assay (ELISA). Therefore, the aim of this study was to evaluate the performance of RPR and ELISA with the Treponema pallidum hemagglutination assay (TPHA - the gold standard) for the detection of syphilis in blood donors. Methods In this cross-sectional study, 1524 consecutive whole blood donors were screened from April to October 2022. All blood samples collected during the study period were tested by RPR, ELISA and the TPHA and the results obtained were compared. Results The seroprevalence of syphilis in blood donors in this study was 0.06% by RPR and 0.72% by ELISA and TPHA. On considering ELISA and the TPHA as the gold standard, ELISA had comparable sensitivity (100%), a higher specificity (100% vs. 99.34%), a higher positive predictive value (PPV - 100% vs. 9.1%) and no biological false positive/false negative results (0 vs. 10 false negatives) when compared to RPR. Conclusion ELISA performed better as a screening assay than RPR in the detection of syphilis in blood donors, which is in agreement with the WHO recommendations for syphilis testing in blood donors with low prevalence.

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Abstract Background Systemic and pulmonary coagulopathy and inflammation are important characteristics of transfusion-related acute lung injury (TRALI). Whether microparticles that accumulate in transfused red blood cell concentrates (RBCs) have proinflammatory and procoagulant potential and contribute to adverse reactions of RBC transfusions is unclear. Aim To investigate the ability of microparticles in stored RBCs to promote thrombin generation and induce human pulmonary microvascular endothelial cell (HMVEC) activation and damage. Methods The number and size of microparticles were determined by flow cytometric and nanoparticle tracking analyses, respectively. Thrombin generation and the intrinsic coagulation pathway were assayed by a calibrated automated thrombogram and by measuring activated partial thromboplastin time (aPTT), respectively. The expression of ICAM-1 and the release of cytokines by endothelial cells were detected by flow cytometric analyses. HMVEC damage was assessed by incubating lipopolysaccharide-activated endothelial cells with MP-primed polymorphonuclear neutrophils (PMNs). Results The size of the microparticles in the RBC supernatant was approximately 100-300 nm. Microparticles promoted thrombin generation in a dose-dependent manner and the aPTT was shortened. Depleting microparticles from the supernatant of RBCs stored for 35 days by either filtration or centrifugation significantly decreased the promotion of thrombin generation. The expression of ICAM-1 on HMVECs was increased significantly by incubation with isolated microparticles. Furthermore, microparticles induced the release of interleukin-6 (IL-6) and interleukin-8 (IL-8) from HMVECs. Microparticles induced lipopolysaccharide-activated HMVEC damage by priming PMNs, but this effect was prevented by inhibiting the PMNs respiratory burst with apocynin. Conclusion Microparticles in stored RBCs promote thrombin generation, HMVEC activation and damage which may be involved in TRALI development.

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Abstract Background Regarding the close association between neonatal hyperbilirubinemia and occurrence of pathological jaundice as a cause of neurotoxicity and kernicterus, the present study aimed to evaluate the use of intravenous immunoglobulin (IVIG) in neonates with hyperbilirubinemia. Methods A retrospective case-control study of blood group O mothers and their ABO and Rh newborns was conducted. Medical records that included total serum bilirubin levels of 79 patients with hemolytic disease of the newborn (HDN) from between 2017 and 2020 were reviewed. Neonates who were eligible to receive immunoglobulin based on the American Academy of Pediatrics (AAP) guidelines were classified as cases and the rest were included as the Control Group. Results The mean total bilirubin in relation to hemoglobin levels in IVIG-treated neonates was significantly lower than in non-IVIG-treated neonates (13.98 ± 4.23 mg/dL versus 16.61 ± 2.68 mg/dL; p-value = 0.002). Although females had longer hospitalizations in both IVIG-treated (3.81 ± 1.28 versus 3.54 ± 1.30 days; p-value = 0.509) and non-IVIG-treated (3.43 ± 0.811 versus 3.19 ± 0.75 days; p-value = 0.361) groups compared to males, this difference was not significant between the groups. Although four neonates with ABO incompatibility required packed red blood cells, all infants were managed medically and no deaths occurred during the course of treatment. Moreover, no exchange transfusion or adverse effects of IVIG were observed. Conclusion The results from the present study revealed that IVIG administration is a useful procedure for the management of bilirubin encephalopathy with greater opportunity to reduce exchange transfusion requirements for neonatal hyperbilirubinemia.

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Abstract Objective To clinically and laboratory characterize patients with a positive direct antiglobulin test (DAT) treated at the Hospital das Clínicas of the Federal University of Goiás (HC-UFG). Methods A retrospective, descriptive, cross-sectional study was carried out collecting data from medical records of patients with a positive DAT who were treated at HC-UFG between August 2021 and August 2022. Results Eighty-four patients with positive polyspecific DAT results were screened in the clinical laboratory. Fifty-four patients had a laboratory profile compatible with autoimmune hemolytic anemia (AIHA), however, among these, 16 patients already had a diagnosis of AIHA in their medical records. The most common symptoms present among AIHA patients were pallor, asthenia, fatigue and dyspnea. For the remaining patients, the most common symptoms were severe thrombocytopenia, anemia, renal dysfunction, fever, myalgia, headache, thrombosis, asthenia, hematuria and joint pain. Only one patient had primary AIHA, that is, he had no evident underlying disease. The majority of AIHA patients (75 %) underwent corticosteroid therapy with 60 % having a positive response. For patients without AIHA, prednisone was the most frequently prescribed medication in 17 (25 %) patients, followed by hydroxychloroquine (14 patients - 20.1 %). Conclusion It is essential to evaluate patients with positive DAT in detail in order to understand the real clinical case. The DAT serological result alone does not arrive at a conclusive diagnosis of AIHA, and so it must be evaluated in conjunction with both clinical data and other laboratory tests, such as hemoglobin concentration and hemolysis tests (reticulocytes, lactate dehydrogenase and/or haptoglobin).

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Abstract Introduction Sub-Saharan Africa struggles continuously with insufficient resources and inadequate infrastructure that hinder the establishment of a safer blood supply despite improvements in transfusion safety over recent decades. This study aimed to evaluate the impact of the chemiluminescence technique in combination with immunoenzymatic and immunochromatographic tests for viral marker screening of hepatitis B (HBV), hepatitis C (HCV) and human immunodeficiency virus (HIV) in donated blood in a country of sub-Saharan Africa. Method This study was conducted in a population of 113,406 blood donors at the National Centre of Blood Transfusion in Senegal. The data were obtained from the ‘INLOG’ software and donor registers. Statistical analyses used Excel 2010 and Epi Info v6. Screening for HBsAg viral markers, anti-HCV Ab, HIV p24 Ag, anti-HIV1 and anti-HIV2 antibodies were first carried out using the chemiluminescence technique. Blood donations screened positive for HBV or HCV were retested in a second chemiluminescence equipment. HIV-positive donations and their controls were subjected to solid phase immunochromatographic and indirect enzyme immunoassay techniques. Results The prevalence among donors of HBV was 8.39 %, 0.56 % for HCV and 0.18 % for HIV. Of the donors tested positive for HIV in screenings and in doubled-controls, only 61.54 % were confirmed by the alternative tests; 34.02 % were negative and 4.44 % discordant between the three techniques. Conclusion This study shows the importance of introducing the chemiluminescence technique in association with serological screening of transfusion-transmitted viruses to improve blood supply safety in low-income countries.

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Abstract Background Platelet additive solutions (PASs) improve the efficacy of stored platelets. Oxidative stress causes storage lesions and platelet functions deteriorate. Studies assessing the influence of oxidative stress on platelets stored in PASs are limited. This study compares variations in platelets in different storage solutions (SSP+, PAS-G and Tyrode's buffer). Methods Platelets isolated from the blood of Wistar rats were resuspended in SSP+, PAS-G and Tyrode's buffer and stored for seven days at 22 °C. The markers of platelet metabolism, function, oxidative stress, antioxidant status and viability were analyzed on Days 1, 3, 5 and 7 of storage. Main results SSP+ is associated with platelet function, viability and antioxidant defenses (SOD, CAT and GSH); it decreased primary lipid peroxidation products and maintained the susceptible protein groups in reduced state. Platelet function, antioxidant defenses such as SOD and GSH improved, and lipids and thiols were protected from oxidation in PAS-G. SOD and GSH increased, and lipids and thiols were preserved in Tyrode's buffer. Conclusion SSP+ and PAS-G are more effective in maintaining platelet efficacy till Day 7 compared to Tyrode's buffer. Thus, PAS-G and SSP+ are better than Tyrode's buffer in terms of platelet responses to oxidative stress during storage. This is the first comparative account on the influence of PASs (SSP+, PAS-G and Tyrode's buffer) on platelets in altering oxidative stress. It provides a comprehensive view of the differential responses of platelets in PASs.

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Abstract Background Emergency transfusion may require the availability of O-negative red blood cell concentrates without pre-transfusion testing. At the Cliniques Universitaires Saint-Luc, the emergency department was used to having access to two decentralized O-negative red blood cell concentrates. This study aims to analyze the consumption of O-negative red blood cell concentrates in emergency situations both before and after the implementation of a novel strategy aiming at optimizing stocks. This strategy provides a combined allocation of one unit of O-positive red blood cell concentrate and one unit of O-negative red blood cell concentrate decentralized in the emergency department and reserve the transfusion of the negative unit only to under 45-year-old women and under 20-year-old men. Materials and Methods A retrospective study was conducted of the transfusion and medical records of all patients who received immediate transfusions in the emergency department without pre-transfusion testing between 2008 and 2022. Results A total of 193 patients received O red blood cell concentrates without pre-transfusion testing in emergency situations between 2008 and 2022. During the first 24 h of hospitalization, 354 O-negative units were transfused. Mean ratios of number of O-negative bags between 2008 and 2020 was 1.98 unit/patient. After implementation of the new strategy, the ratio in 2021 was 1.46 unit/patient and drastically decreased in 2022 to 0.79 unit/patient. Conclusion In situations of emergency, allocating O-negative units only for women younger than 45 years and men younger than 20 years could have saved 85% of O-negative red blood cell concentrates transfused (303/354) yet balancing the immunological risk. Limiting the number of delocalized units of O-negative red blood cell concentrates in the emergency department seems to lower O-negative consumption. With this strategy, the units spared could have been transfused to patients with greater needs (e.g., sickle cell patients or chronically transfused patients).

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Abstract Background and Objectives The identification of platelet antibodies is essential for diagnosing and managing conditions such as fetal and neonatal alloimmune thrombocytopenic purpura, post-transfusion purpura, and immune platelet refractoriness. Monoclonal antibody immobilization of platelet antigens (MAIPA) is the standard method for detecting anti-human platelet antigen (HPA) antibodies, while the detection of anti-HLA antibodies once relied on the complement-dependent cytotoxicity method, however advanced technologies such as enzyme-linked immunosorbent assay and Luminex have significantly improved sensitivity and accuracy in identifying these antibodies. Flow cytometry-based techniques (platelet immunofluorescence test - PIFT) and Luminex platform-driven microsphere-based multiplex assays (Pak-Lx) are widely employed in platelet immunology laboratories owing to their remarkable flexibility and versatility. The present study compared the sensitivity, specificity, and concordance of these different serological techniques used in platelet antibody identification. Material and Methods One hundred serum samples from patients suspected of immune-mediated platelet disorders were examined. Initially, the samples underwent testing using the MAIPA method. Subsequently, the results were compared with three alternative methods: PIFT and microsphere-based multiplex assays for both HLA and HPA antibodies. Results Pak-Lx demonstrated a 94 % agreement with MAIPA, while PIFT had 88 % agreement for HPA antibodies. For HLA antibody detection, Pak-Lx versus DLX had 75 % concordance, MAIPA versus DLX showed 77 %, and PIFT versus DLX displayed an 81 % concordance rate. Remarkably, there were no significant differences in concordance levels between Pak-Lx and PIFT compared to MAIPA and DLX for anti-HPA and HLA antibodies, respectively. Conclusion This study found no significant differences in concordance among the tested assays for detecting anti-HPA and anti-HLA antibodies. These data suggest that no single method can detect all clinically important antibodies. Therefore, it is advisable that each laboratory develops customized protocols based on their expertise and employs complementary methods for comprehensive patient assessments.

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Abstarct Introduction Therapeutic erythrocytapheresis has some advantages over therapeutic phlebotomy, the standard treatment for cytoreduction in polycythemia and hemochromatosis. Erythrocytapheresis can be performed on different cell separators, each with its own characteristics. We present our experience of therapeutic erythrocytapheresis in the treatment of polycythemia and hemochromatosis with an analysis of the performance of cytoreduction, and a comparison between the characteristics of intermittent- and continuous-flow cell separators. Material and methods During a 20-year period, 1731 procedures were performed in 125 patients, 1634 (94.4%) with a Haemonetics MCS+ separator and 97 (5.6%) with a Spectra Optia system device. The performance of cytoreduction using the Haemonetics MCS+ separator was analysed in 442 procedures performed in 56 patients and the performance of the two apheresis devices was compared. Results Haemoglobin (Hb) and haematocrit (Hct) values were significantly reduced after erythrocytapheresis with the Haemonetics MCS+ device (Hb: 18.69%; Hct: 18.73%; p-values both <0.001). The reductions of Hb and Hct were significantly higher in the Haemonetics MCS+ procedure (p-value <0.001), but the Spectra Optia procedure depleted a significantly higher RBC volume (495 mL versus 442 mL) in a shorter time (18 min versus 36 min). Conclusion Both the Haemonetics MCS+ and Spectra Optia systems proved to be highly efficient and safe in RBC cytoreduction with short procedure times. Erythrocytapheresis reduces the frequency of necessary procedures thereby justifying its therapeutic use especially in eligible patients of working age.

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Abstract Introduction The incidence of feto-maternal complications is high in women with sickle cell disease. The paucity of high-quality evidence has led to conditional recommendations for transfusional support in pregnant patients. This study aimed to assess if scheduled partial red cell exchanges impact pregnancy outcomes in sickle cell disease patients. Methods Forty-seven pregnancies were divided into two groups based on whether patients received scheduled partial red cell exchanges. Collected data included demographics, laboratory values, number of hospital visits, and prenatal/perinatal/postnatal outcomes. Data were analyzed using descriptive statistics, t-test, Chi-square and Fisher's exact tests, and binary regression. Results The mean age was 25.09 ± 4.39 years. Of 47 patients, 14 (29.8%) received scheduled red cell exchanges with 78.6% compliance with no evidence of alloimmunization. This procedure during pregnancy was associated with fewer admissions for pain crises (p=0.032), higher gestational age at delivery (p=0.007), and a lower incidence of neonatal intensive care admissions (p=0.011; odds ratio: 0.071; 95% confidence interval: 0.008-0.632). Logistic regression did not show any significant associations. Conclusions Sickle cell disease patients with complications in previous pregnancies, including high hospitalization/admission rates and preterm deliveries, could benefit from scheduled partial red cell exchanges or simple transfusions. Further research is needed to guide clinical practice pertaining to transfusional support in pregnant patients with sickle cell disease.

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Abstract Objectives This study aimed to determine the true frequency of weak ABO subgroups and to investigate the serological characteristics of various subgroup alleles in the Chinese population. Methods A total of 2,945,643 blood samples were collected from January 2009 to December 2017. After initial screening and re-examination using automatic blood group analyzers, all ABO-discrepant samples were confirmed by standard serological analysis and molecular detection by DNA sequencing. The true frequency of weak ABO subgroups was determined by the number of ABO subgroup donors and the missed detection rate. The ABO antigen expressions corresponding to subgroup alleles were analyzed by the agglutination intensity of red blood cells. Results The detection rate of ABO subgroups was 0.031 % (927/2,945,643). Considering the missed detection rate (27.81 %), the true frequency of ABO subgroups in the Chinese population was 0.044 %. The three most common genetic variations among blood donors in Shanghai were BA.04, BW.12 and BA.02. BW.03 showed the weakest B antigen expression (6.00 ± 1.97) and Bvar-1 the strongest (9.20 ± 1.10). Conclusion Many ABO subgroups were missed. BA.04 was found to be the most common subgroup allele in the Chinese population. Different ABO subgroup alleles exhibit different ABO antigen expression patterns.

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Abstract Background Alternative approaches have been proposed to ensure a safe and equitable screening process for blood donation that treats all people equally, regardless of gender identity or sexual orientation. The terms ‘neutral approach’ and ‘individualized risk assessment’ have been used to describe this goal. To facilitate research and implementation of these concepts in blood donation contexts and health services in Brazil, we propose a Portuguese version of the ‘for the assessment of individualized risk screening criteria’ (FAIR) screening criteria. Methods The FAIR screening criteria are 12 questions that assess sex, sexuality, ethnicity, and the extent to which participants engaged in each targeted sexual behavior. The aim of FAIR is to reduce error while increasing reliable and accurate reporting of sexual behaviors associated with both objective and subjective estimates of infection risk. The FAIR screening criteria were translated and cross-culturally adapted using a systematic approach with standardized procedures appropriate for adapting instruments that track behaviors. Results A version that is appropriate for use with the Brazilian population was produced employing the following steps: expert translations, harmonization, consensus version, expert back-translation, revision, panel of experts, cognitive interviewing, and finalization. Conclusion The Portuguese version of FAIR was proposed, and because of its straightforward, simple language and focus on specific and frequent behaviors in some populations, it has the potential to be used in a variety of contexts involving the screening of high-risk sexual behavior in Brazil.

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Abstract Introduction Alloimmunization and transfusion reactions underscore the crucial role of precise immunohematological techniques to enhance safety in transfusion. This study aims to determine the frequency of alloimmunization in patients treated at a Brazilian university hospital, investigate demographic, clinical, and epidemiological characteristics of patients with positive irregular antibody screening, as well as to assess the frequency of erythrocyte antigens and anti-erythrocyte antibodies in the population. Materials and methods This retrospective observational study included all irregular antibody-positive patients treated at the transfusion service of Hospital de Clínicas of the Federal University of Uberlandia between January 2019 and December 2020. Results Of the 201 irregular antibody-positive patients, alloimmunization was more common in women (64.2%) than in men (35.8%). Blood groups A (39.8%) and O (38.8%), and Rh positive samples (69.1%) predominated, and about half (48.2%) of the patients were transfused for preoperative procedures. The most frequently found clinically significant alloantibodies were anti-D (27.2%), anti-E (15.0%), and anti-Kell (11.5%). Of the patients, 30.6% had multiple antibody associations, with anti-D and anti-C being the most common combination. Erythrocyte immunophenotyping was performed for 76 patients with the most frequent antigens detected being e (100%), c (86.8%), and C (40.8%). Among the 14 pregnant women evaluated, most were multiparous, 85.7% had anti-D as the most prevalent antibody, and had the A-negative blood type (33.3%). Conclusion Alloantibody screening and identification associated with erythrocyte immunophenotyping are necessary for a better understanding of the alloimmunized population, ensuring greater safety and efficacy of transfusion therapy in the hospital setting.

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Abstract Introduction and Objective Flow Cytometry (FC) is one of the techniques, which allows the identification and characterization of platelets. The detection of absent or reduced expression of the glycoproteins is the main objective of this technique. Abnormalities of glycoproteins lead to hemorrhagic syndromes. Among the main diseases, the Bernard-Soulier syndrome (BSS) and Glanzmann thrombasthenia (GT) stand out. We aimed to show a FC-based platelet assessment test for diagnostic use, which measures the expression of markers in normal patients, and evaluate these markers in patients with platelet disorders. Methods We examined a control group of 41 healthy adults to establish reference values and assess the variability of the relative expression of platelet markers and subsequently compared these findings to those of 30 patients with suspected platelet dysfunctions. We determined the mean fluorescent intensity (MFI) of the expressed parameters by FC using CD41, CD42a, CD42b and CD61 and SSC/FSC platelet-gated cells. Results We determined our baseline panel of markers and compared them to suspected platelet dysfunctions. Patients with suspected BSS presented increased levels of the MFI for the GPIIIa (CD61) and GPIIb (CD41). They showed significantly reduced levels of the GPIb (CD42b) and GPIX (CD42a). Patients with suspected GT showed normal expression of the GPIX (CD42a), increased expression of the GPIb (CD42b) and reduced levels of the GPIIIa (CD61). In this case, with reduced levels of only one marker, the GPIIb (CD41), values showed normal expression. Conclusions We describe the FC assay to support the diagnosis of different platelet disorders. Our study made it possible to implement a technique that brought benefits to care.

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Abstract Introduction Despite knowledge advances on extramedullary haematopoiesis (EMH) in thalassemic patients, the real picture remains an open issue. Objectives To assess EMH prevalence in patients with thalassemia major (TM) and intermedia (TI), to describe magnetic resonance imaging (MRI) findings and to explore clinical risk factors. Methods In this cross-sectional study, images and clinical records of 184 consecutive patients with thalassemia who underwent T2* MRI between 2004 and 2011 were reviewed. Association of EMH with survival was investigated for patients with available follow-up charts. Results EMH was detected in 16/168 (9.5%) patients with TM (aged 19-49 years) and in 3/16 (18.8%) with TI (aged 36-41 years). Most (88%) had paravertebral thoracic and/or abdominal masses. Age was significantly associated with EMH risk (hazard ratio, [HR] 1.10/year; confidence interval [CI]: 1.03-1.18; p-value < 0.001), while lower pancreatic iron content by T2*MRI (HR: 0.94/ms; CI: 0.89-0.99; p-value = 0.049) was a protective factor. Estimated survival rate was superior for EMH-positive (n = 19) when compared to EMH-negative patients (n = 75) (p-value = 0.013). Conclusions The prevalence of EMH was 10.3% (19/184), presented mainly as tumoral masses of 3 to 10 cm. Age was a risk factor for EMH development, while lower pancreatic iron might be a protective factor in this cohort.

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Abstract Immune thrombocytopenia (ITP) is an acquired bleeding disorder observed in the clinical practice. Little is known about its epidemiology in Brazil. The present study was conducted at a hematology referral center which covers a population of over 8 million in 184 municipalities in the state of Ceará. The purpose of this study was to draw a demographic profile of adult ITP patients with regard to sex, age, geographical origin and distribution across the state, and the proportion of secondary ITP. Following ethics committee approval, information was collected with an ad hoc instrument. The sample consisted of 187 adult ITP patients attending the Walter Cantídio University Hospital in 2015. The median follow-up time was 67 months (range: 1 month to 29 years). Female sex (n = 154; 82.35 %) was strongly prevalent in all age brackets, with an overall female/male ratio of 4.7:1. The median age was 41 ± 16.1 with an interquartile range of 29-55.5 years; there was no difference between the genders. Secondary ITP (18/187; 9.6 %) displayed a bimodal distribution and a linear increase between 38 and >68 years of age. The results of this survey on the epidemiology of ITP in Brazil suggest that ethnic and geographical factors may have a great impact on age and sex distribution and on the distribution of secondary ITP.

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Abstract Introduction The Hb Deer Lodge (β2 His>Arg; HBB:c.8A>G) is a structural hemoglobin variant described in some populations around the world, characterized by increased oxygen affinity, but does not confer clinical symptoms to its carriers. The coinheritance of the Hb Deer Lodge with the most common hemoglobin variant, Hb S, has been reported only once; however, functional data were not described. Here we show a case of the Hb S and Hb Deer Lodge carrier in heterozygosity. Methods The Hb S and Hb Deer Lodge association was identified by High-Performance Liquid Chromatography (HPLC), reverse phase HPLC and the β globin gene sequencing. The functional characterization of this interaction was obtained using the O2 dissociation curve, determination of the cooperativity between the globin chains and the Bohr effect in the presence and absence of organic phosphates. Results When the Hb S and Hb Deer Lodge were associated, there was a decrease in cooperativity, no significant changes in oxygen affinity and no significant Bohr effect changes. Conclusion Despite these genetic variations, the carrier showed no hematological alterations and no clinical symptoms, possibly due to the high oxygen affinity of the Hb Deer Lodge, which interferes with the Hb S polymerization.

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Abstract Objective This study aims to evaluate the markers of tubular phosphate handling in adults with sickle cell anemia (SCA) and the influence of hydroxyurea (HU), the degree of anemia and Hb F concentration on these markers. Methods Eighty-eight steady state SCA patients in outpatient follow-up in Fortaleza, Ceara, Brazil and 31 healthy individuals were included in this study. Vitamin D (25OHD) was measured by enzyme-bound fluorescence assay, intact parathyroid hormone (iPTH) by electrochemiluminescence, and serum and urinary phosphate and creatinine by colorimetric methods. Details of Hb F and HU use were obtained from clinical records. Tubular reabsorption of phosphate (TRP) and maximum tubular reabsorption of phosphate (MTRP) were calculated. SCA patients were stratified according to the use of HU, degree of anemia and percentage of Hb F. The significance level was set for p-values <0.05. Results Compared to controls the 25OHD level (25 ± 11 vs. 30 ± 9 pg/mL) was lower in SCA, while serum phosphate and MTRP were higher (3.86 ± 0.94 vs. 3.46 ± 0.72 and 3.6 ± 1.21 vs. 3.21 ± 0.53, respectively). There was no significant difference in iPTH, TRP and phosphaturia. Serum phosphate showed correlation with TRP (r = 0.32; p-value = 0.008) and MTRP (r = 0.9; p-value <0.001) in SCA. Patients taking HU, especially those with Hb F >10 % presented reduced serum phosphate levels, and TRP and MTRP rates. Those with mild anemia presented reduced serum phosphate levels and MTRP rates. Conclusion Serum phosphate levels and renal phosphate reabsorption rate were increased in SCA. HU use, high Hb F concentration and total Hb were associated with better control of tubular phosphate handling markers.

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Abstract Background Thrombotic microangiopathies (TMA) are a group of disorders with overlapping clinical features that require urgent intervention. Treatment is based on the recognition of the TMA type, which is often challenging. The aim of this study was to identify specific HLA associations with different TMA types to aid rapid diagnosis and appropriate treatment, since the HLA assay can be completed within five hours. Methods All 86 consecutive patients who presented to the University of Arkansas for Medical Sciences between May 2013 and January 2021 with a presumptive diagnosis of TMA were included in this study. HLA typing was performed and correlated with other clinical and laboratory studies. Results In comparison with other types of TMA, patients with acquired thrombotic thrombocytopenic purpura (aTTP) showed increased frequencies of HLA-DRB1*11, HLA-DQB1*03:01/19, HLA-DRB1*08 and HLA-DRB3. Combining the presence of these HLA associations with a PLASMIC score of 6 or more achieved a higher positive predictive value (90%) for identifying aTTP than the PLASMIC score alone (69%). In comparison with other TMA types, patients with aTTP showed decreased frequencies of HLA-DRB4, HLA-DRB1*07, HLA-DQB1*02. The HLA-DRB1*07/DQB1*02 was not observed in any aTTP patients (negative predictive value: 100%), and thus the presence of this haplotype essentially rules out aTTP. Further, HLA-DRB1*11/DQB1*03:01/19 was absent in atypical hemolytic uremic syndrome patients. Conclusion HLA alleles can be used as an adjunct for the rapid assessment of TMA and can help to differentiate it from other primary and secondary forms of TMA, allowing for earlier definitive therapy.

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Abstract Background Autoimmune hemolytic anemia (AIHA) is a rare, life-threatening disease in pediatrics. This article describes the clinical features, diagnostic workup, treatment and outcome in patients with AIHA. Method Medical charts of under 18-year-old patients with AIHA treated at a tertiary Brazilian institution from 2006 to 2021 were retrospectively reviewed. Data analysis was primarily descriptive, using medians, interquartile ranges, and categorical variables presented as absolute frequencies. Main results Twenty-four patients (14 female, 10 male) were evaluated in this study. The median age at diagnosis was 5.99 years (range: 0.25-17.1 years) and the median hemoglobin level was 4.85 g/dL (range: 4.17-5.57 g/dL). Most had warm antibodies (83.3 %). Twelve patients (50 %) had known underlining diseases, four (16.6 %) presented with AIHA concomitant with acute infectious diseases and three (12.5 %) had an undetermined post-vaccine association. Steroids and intravenous immunoglobulin were first-line therapy in 23 cases. Seven patients (29.1 %) required second and third-line treatments (rituximab, cyclophosphamide and splenectomy). The median follow-up period was 4.4 years (range: 1.0-6.7 years). Thirteen patients (54.1 %) were discharged, five cases (20.8 %) were lost to follow-up and no patient died. The median age for the six remaining patients was 11.53 years (8.5-14.7) with all of them having complete responses with no further therapies. Conclusion Most cases of AIHA are secondary to an underlying systemic disease or have a possible correlation with infections/vaccines and respond to steroids. The second and third-line therapies for refractory and relapse cases remain a dilemma. A prospective, multicenter study is essential to address the best therapeutic combinations.

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Abstract Background Sickle cell disease (SCD) comprises a heterogeneous group of inherited hemolytic disorders that increases the risk of maternal and perinatal complications due to chronic systemic inflammatory response, endothelial damage and vaso-occlusion. The contribution of genotypes to the severity of outcomes during pregnancy is not completely established. Methods A retrospective study of medical charts was performed to compare maternal and perinatal outcomes in Hb SS, Hb SC disease and sickle-beta thalassemia (Hb Sβ) pregnancies followed at a high-risk antenatal care unit over a 6-year period. A descriptive analysis of morphological findings was performed of the placenta when pathology reports were available. Results Sixty-two SCD pregnant women [25 Hb SS (40 %), 29 Hb SC (47 %) and 8 Hb Sβ (13 %)] were included. Overall, SCD was associated with maternal complications (77 %), preterm birth (30 %), cesarean section (80 %) and a need of blood transfusion. In general there were no statistically significant differences between genotypes. The only significant difference was the hemoglobin level at first antenatal care visit which was lower for the homozygous genotype (7.7 g/dL) compared to Hb SC and Hb Sβ (9.7 g/dL and 8.4 g/dL, respectively; p-value = 0.01). Ten of 15 evaluated placentas showed abnormal morphological findings Conclusion SCD, regardless of the underlying genotype, is associated with increased adverse maternal and perinatal outcomes and placental abnormalities associated with maternal vascular malperfusion.

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Abstract Background Iron overload (IO) is a complex condition in which clinical, behavioral and genetic factors contribute to the phenotype. In multiethnic and non-Caucasian populations, mutations in HFE gene alone cannot explain IO in most of the cases, and additional genetic and environmental factors must be investigated. Bone Morphogenetic Proteins (BMPs) play a central role in iron homeostasis by modulating HAMP transcription through the signaling pathway that includes SMAD and HJV. In this study, we aimed to explore the clinical relevance of BMP6 mutations in a cohort of Brazilian patients with IO. Methods 41 patients with IO were evaluated. Blood samples were collected to analyze BMP6 mutations through New Sequence Generations (NGS). Frequency of variants and mutations were analyzed and correlated with clinical and environmental characteristics. Results We identified BMP6 mutations in three patients with IO. The p.Arg257His mutation was identified in two patients and the p.Leu71Val mutation was identified in one patient. Two of these patients had additional risk factors for IO (HFE mutations and diabetes mellitus). Conclusion BMP6 mutations, when combined to other genetic and clinical risk factors, may contribute to IO. Functional studies and THE evaluation of large cohorts are necessary to fully address BMP6 role in IO.

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Abstract Objective To evaluate the knowledge of pregnant women and the clinical management of hemolytic disease of the fetus and newborn, as well as to describe the gestational profile, risk factors and socio-epidemiological profile of pregnant women treated at two municipal health units in Belém (Pará, Brazil). Methods This was a cross-sectional analytical study, which consisted in the application of questionnaires to pregnant women who underwent prenatal care at the municipal health units. Results A total of 104 pregnant women were evaluated; most were aged between 24 and 29 years old, had high school degrees (38 %), family incomes between 1 and 2 minimum wages (45 %) and blood type O+ (43 %). Regarding the gestational profile, the participants were predominantly in the third trimester of pregnancy (49 %), started prenatal care in the first gestational trimester (81 %) and were primiparous (61 %). Failures in the management of prenatal care were observed, especially with regard to access to information about the disease, since most pregnant women did not receive information about blood incompatibility during prenatal care. This led to limited knowledge about the pathology of the disease evidenced by the fact that most of the correct answers were between Questions 0-4, which were significantly associated with the women's education and income. Conclusions Although hemolytic disease of the fetus and newborn is serious, the pregnant women in this study demonstrated little knowledge about the disease and had inadequate care by health professionals, reinforcing the importance of improving care for women's health and prenatal care.

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Abstract Background Immune thrombocytopenic purpura, a recurrent autoimmune disease, is characterized by thrombocytopenia, purpura and hemorrhagic episodes with the main factor in the pathogenesis of this disease being autoantibodies against platelets. Since the 1950s, first-line treatment has been glucocorticoids that have indirect and direct effects on thrombocytopenia. Although the characteristics associated with the chronicization of immune thrombocytopenic purpura at the time of diagnosis have been investigated in previous studies, no study was found in the literature investigating the relationship between the response to first-line steroid treatment and the course of the disease, the aim of this study. Materials and methods This retrospective, single center study revisited electronic files of patients with a diagnosis of immune thrombocytopenic purpura between September 2012 and September at the Department of Clinical Hematology, Selcuk University Faculty of Medicine 2022. The platelet count had been confirmed by peripheral blood smears of patients with a platelet count ≤30 × 109/L. The bleeding status of patients at the time of diagnosis was evaluated according to the immune thrombocytopenic purpura bleeding score. Patient responses to treatment were categorized in three groups: a platelet count ≤30 × 109/L was defined as no-response, a platelet count of 30-100 × 109/L was defined as partial response, and a platelet count >100 × 109/L was defined as complete response. Subsequently, patients in the partial or complete response groups were divided into two subgroups: patients who remained in remission for less than or more than six months. Results A total of 100 patients were included in the study; 73 % were in the young (19-65 years old) and 27 % in the old (>65 years old) age group. Most of the patients were female (69 %). Forty-one patients were hospitalized without bleeding. The complete response rate to first-line treatment was 61 %. There was no significant difference between the agents given in first-line treatment in terms of response and length of remission. Conclusion The main purpose of immune thrombocytopenic purpura treatment is to prevent severe bleeding rather than bringing the platelet count to normal values. Glucocorticoids, the first step of treatment, provide high response rates. There is no significant difference between glucocorticoid agents in terms of response to treatment and long-term remission. The points to be considered in the selection of glucocorticoid agents are the side effect profiles, ease of administration and individualization of treatment.

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Background Anemia-associated chronic kidney disease increases in more advanced stages with a subsequent acceleration in renal impairment progressing to end-stage renal disease. Although hepcidin and erythroferrone have been described as novel biomarkers of iron metabolism, there is still an area of ambiguity regarding iron utility in anemia-associated end-stage renal disease. Objectives This study aims to determine the correlations between erythropoietin, erythroferrone, and hepcidin-25 in hemodialysis, and to evaluate the clinical utility of the hepcidin-25/erythroferrone ratio as a biomarker of erythropoiesis-stimulating agent effectiveness compared to reticulocyte maturation parameters. Methods Serum erythropoietin, erythroferrone, and hepcidin-25 levels in 35 dialysis-dependent patients on a maintenance dose of a short-acting erythropoiesis-stimulating agent were consequently assessed on Days 0, 5, and 7. The erythropoiesis activity was monitored by measuring the increment in reticulocyte maturation parameters. Results Though the effectiveness of erythropoiesis in these patients was not associated with the hepcidin-25/erythroferrone ratio, it was lower among those with effective erythropoiesis than those with ineffective erythropoiesis. The effective group showed a statistically significant increase in reticulocyte maturation parameters compared to the ineffective group. Conclusions The findings show the pathogenesis of iron homeostasis in hemodialysis, the validity of hepcidin-25/erythroferrone ratio as a biomarker of erythropoiesis-stimulating agent effectiveness, and the advantageous monitoring of reticulocyte maturation measures to improve management of anemia-associated chronic kidney disease.

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Abstract Introduction Surgeries are implicated in the development of anti-factor VIII (FVIII) neutralizing antibodies (inhibitors) in hemophilia A individuals with immune tolerance induction (ITI) treatment being the recommended therapy to eradicate these inhibitors. We evaluated the association of surgical procedures performed during ITI and treatment outcome. Methods Patients were treated according to the Brazilian ITI Protocol with outcomes being defined as successful (i.e., recovered responsiveness to exogenous FVIII) and failed (i.e., unresponsiveness to exogenous FVIII thus requiring bypassing agents for bleeding control). Surgical procedures during induction therapy were managed following international recommendations. Results Treatment success rate was 68.7 % in 163 patients; 33 (20.2 %) were submitted to 43 (96 %) minor and two major surgeries. Personal, hemophilia, inhibitor, and treatment characteristics were similar between patients submitted to surgical procedures or not while on ITI; the success rates were 72.7 % and 67.7 % (p-value = 0.577), respectively. Conclusion No association was found between having a minor surgical procedure and ITI treatment outcome.

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Abstract Background As the diagnosis of Pediatric venous thromboembolism has dramatically increased in recent decades, this study aims to evaluate these patients, determining the incidence and describing their biological and clinical characteristics. Methods An observational, cross-sectional study was conducted at a Brazilian quaternary hospital between January 2022 and February 2023. Under 18-year-old hospitalized patients with a confirmed diagnosis of venous thromboembolism were included, while those with arterial or chronic thrombosis were excluded. Data on biological and clinical characteristics, diagnosis and treatment were evaluated. A descriptive data analysis was performed and the incidence of hospital-associated thrombosis was calculated. Results Thirty-nine pediatric patients were evaluated. The incidence of hospital-associated thrombosis was 19.9 cases per 10,000 pediatric hospitalizations. Median age at diagnosis was four months (range: 12 days-17 years). Most of the patients (66.7%) were asymptomatic, with venous thromboembolism being diagnosed incidentally. In all cases, at least one risk factor was identified and in 74.6% of cases four or more factors were present. The principal risk factors were the presence of a central venous catheter (89.7%) and infection (89.7%). Thrombogenic comorbidities, particularly congenital heart disease, were present in 48.7% of patients. Conclusions The incidence of venous thromboembolism found in the present study was lower than rates reported in developed countries. The principal characteristics of this sample were a greater frequency of central venous catheter and infection as risk factors, and the fact that the cases consisted mainly of newborns and individuals with heart disease.

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Abstract Background The decline in the mental well-being of young adults following an episode of venous thromboembolism may be related to the uncertainty of long-term health and fear of recurrence. In recent years, post-pulmonary embolism syndrome has gained acceptance, however, less attention has been given to the psychological impact on young patients after venous thromboembolism. This study explores the prevalence, type, and severity of psychological disorders of patients following venous thromboembolism. Methods A retrospective observational cohort study was performed of over 18-year-old patients diagnosed with venous thromboembolism followed in the Vascular Medicine Service at Hospital Privado de Córdoba, Argentina from July 2020 to October 2021. Due to the COVID-19 pandemic, virtual interviews were conducted using two pre-established questionnaires administered by the same psychiatrist. The first questionnaire gathered personal data, clinical history, and mental health information, while the second, evaluated mood disorders using the Mini International Neuropsychiatric Interview. Patients with a positive MINI score underwent further assessment using the Hamilton Scale. Patients were considered young if ≤45 years. Results A total of 50 patients were assessed, 56 % were women, and 54 % were ≤45 years. Major depression was documented in 11 (22 %) patients, eight (72 %) in the younger group, and three (28 %) in the older group. Eight (16 %) patients had an anxiety disorder, four in the younger group, and ten (20 %) patients had post-traumatic stress disorder, seven (70 %) of the younger patients. Generalized anxiety disorder was identified in 20 (40 %) patients with similar proportions in both groups. Conclusion Psychological and emotional symptoms are common following an episode of venous thromboembolism. Post-traumatic stress disorder and depression appear to be numerically more prevalent in the young.

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Objective This study aims to identify lung ultrasound (LUS) findings associated with acute chest syndrome (ACS) at the time of admission and 24-48 h later, to compare these to chest radiography (CXR) findings and to establish a score to predict the development of this pulmonary complication in sickle cell disease (SCD) children Methods A prospective observational study of SCD children presenting signs or symptoms of ACS evaluated by LUS and CXR at admission and 24-48 h later. A score was conceived to predict the evolution of ACS during hospitalization based on ultrasonographic findings. Results Seventy-eight children were evaluated; 61 (78.2 %) developed ACS. A score greater than one at admission showed sensitivity, specificity, accuracy, and positive predictive value (PPV) of 75.4 %, 88.2 %, 78.2 %, and 95.8 %, respectively to predict ACS, while only 32 (52.5 %) CXR showed alterations. The development of ACS during hospitalization was unlikely for a score of zero and very likely for a score greater than one at admission. Regarding follow-up exams, a score greater than one showed sensitivity, specificity, accuracy, and PPV of 98.4 %, 76.5 %, 93.6 %, and 92.8 %, respectively to predict the development of ACS. ACS development was very unlikely for a score of zero and very likely for a score greater than zero in the follow-up. Conclusion LUS is an effective tool to assess risk for the development of ACS in SCD children with clinical suspicion.

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Abstract Background Hemolytic disease of the fetus and newborn is a public health problem caused by maternal-fetal incompatibility; no prophylaxis is available for most alloantibodies that induce this disease. This study reviews the literature regarding which antibodies are the most common in maternal plasma and which were involved in hemolytic disease of the fetus and newborn. Method Seventy-five studies were included in this review using a systematic search. Two independent authors identified studies of interest from the PubMed and SciELO databases. Main results Forty-four case reports were identified, of which 11 babies evolved to death. From 17 prevalence studies, the alloimmunization rate was 0.17 % with 161 babies receiving intrauterine transfusions and 23 receiving transfusions after birth. From 28 studies with alloimmunized pregnant women (7616 women), 455 babies received intrauterine transfusions and 21 received transfusions after birth. Conclusion Rh, Kell, and MNS were the commonest blood systems involved. The geographical distribution of studies shows that as these figures vary between continents, more studies should be performed in different countries. Investing in early diagnosis is important to manage the risks and complications of hemolytic disease of the fetus and newborn.

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Abstract Sickle cell anemia is a hereditary disease caused by sickle-shaped red blood cells that can lead to vaso-occlusive crises. Treatment options are currently limited, highlighting the need to develop new clinical approaches. Studies demonstrated that elevated levels of fetal hemoglobin (Hb F) are associated with a reduction of mortality and morbidity in sickle cell anemia patients. In light of this, researchers have been trying to elucidate the transcriptional regulation of Hb F to develop new therapeutic interventions. The present study aimed to present the main transcription factors of Hb F and discuss the clinical feasibility of these molecular targets. Two search strategies were used in the PubMed, SciELO, and LILACS databases between July and August 2023 to conduct this review. Manual searches were also conducted by checking references of potentially eligible studies. Eligibility criteria consisted of clinical trials and cohort studies from the last five years that investigated transcription factors associated with Hb F. The transcription factors investigated in at least four eligible studies were included in this review. As a result, 56 eligible studies provided data on the BCL11A, LRF, NF-Y, GATA1, KLF1, HRI, ATF4, and MYB factors. The studies demonstrated that Hb F is cooperatively regulated by transcription factors with the BCL11A factor appearing to be the most specific target gene for γ-globin induction. Although these data are promising, there are still significant gaps and intervention limitations due to the adverse functions of the target genes. New studies that clarify the aspects and functionalities of Hb F regulators may enable new clinical approaches for sickle cell anemia patients.