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ABSTRACT Introduction: As coronavirus disease-2019 (COVID-19) spread worldwide and social restrictions were intensified, difficulties in blood supply were expected to result in a shortage of blood donors, logistic issues and a change in blood consumption. Consequences could be detrimental to the meeting of the blood supply demand, especially in a decentralized blood bank in the State of São Paulo responsible for providing blood to more than 100 hospitals, mostly of the public health system. Aiming to minimize negative effects and focusing on maintenance of the blood supply, a different approach was discussed and adopted. Materials and methods: Briefly, strategies were related to monitoring and promoting measures to achieve a positive RBC unit balance. Thus, the number of donors, transfusions, RBC unit inventory, RBC unit loss and RBC units within up to 5 days from the expiration date were evaluated. Results: Several strategies were adopted to ensure sufficient availability of RBC units: blood donation was improved with social media and extra blood collections, a restrictive transfusion protocol was implemented, a new logistic process to use RBC units closer to the expiration date was established and non-isogroup transfusions were avoided. Conclusion: Altogether, described strategies were crucial to optimize blood storage during the pandemic. Investing in monitoring and logistics contributed to a positive RBC unit balance and conserving these strategies could be useful.

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ABSTRACT Background: The level of satisfaction of donors with the blood donation system is an important factor which influences their intent to return for future donation. A 25-item questionnaire on the satisfaction of blood donors has been recently proposed to assess the donor satisfaction with the service provided by a blood bank. The objective of this study was to present a proposal for application and interpretation of a BDSQ considering a sample of blood donors of a Brazilian blood bank. Methods: A sample of 1019 blood donors at the Regional Hemotherapy Center of Franca, Brazil, answered the BDSQ. A top-box analysis was used to assess the associations between the variables characterizing the profiles of blood donors and their overall satisfaction with the service provided by the blood bank. We proposed the use of a performance-importance plot (PIP) for the interpretation of the 25 items of the BDSQ. Results: Older donors are more likely to report lower overall satisfaction with the service provided by the blood bank. PIP allows us to classify the items of BDSQ into longand short-term improvement opportunities as well as into motivational and hygiene factors. Motivational factors (i.e. attributes that promote overall satisfaction when present) tend to be associated with the treatment offered by the blood bank staff. Donors seem to give more importance to the interpersonal aspects than to the physical structure of the service and other aspects of convenience. Conclusions: Top-box analysis and PIP proved to be efficient strategies to interpret the results of the BDSQ.

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Abstract Introduction: Sickle cell anemia (SCA) is a Mendelian disorder with a heterogeneous clinical course. The reasons for this phenotypic diversity are not entirely established, but it is known that high fetal hemoglobin levels lead to a milder course of the disease. Additionally, genetic variants in the intergenic region HBS1L-MYB promote high levels of fetal hemoglobin into adulthood. Objective: In the present study, we investigated the HMIP1 C-839A (rs9376092) polymorphism, located at the HBS1L-MYB intergenic region block 1, in SCA patients. Method: We analyzed 299 SCA patients followed in two reference centers in Brazil. The HMIP1 C-839A (rs9376092) genotypes were determined by allele specific polymerase chain reactions. Clinical and laboratory data were obtained from patient interviews and medical records. Results: The median fetal hemoglobin levels were higher in patients with the HMIP1 C-839A (rs9376092) AA genotype (CC = 6.4%, CA = 5.6% and AA = 8.6%), but this difference did not reach significance (p = 0.194). No association between HMIP1 C-839A (rs9376092) genotypes and other clinical and laboratorial features was detected (p > 0.05). Conclusion: In summary, our data could not support the previously related association between the HMIP1 C-893A (rs9376092) polymorphism and differential fetal hemoglobin levels.

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Abstract Introduction: To date, many studies have validated the Hematopoietic Cell Transplantation Specific Comorbidity Index (HCT-CI) scoring system in allogeneic hematopoietic stem cell transplantation (allo-HSCT), but studies from developing countries remain scarce. Objective: The aim of this study was to evaluate and categorize Mexican patients using the HCT-CI at a referral center. Methods: One hundred and nineteen consecutive patients undergoing allo-HSCT at the National Institute of Medical Sciences and Nutrition in Mexico City were included. Patients were classified according to the HCT-CI scores. Results: The median age was 31 years and most were males (56%). Most patients had hematological malignancies (73%) and a low HCT-CI score (72%). The non-relapse mortality and survival were predicted according to the score. Conclusions: This is one of the few studies to evaluate the HCT-CI in adults with HLA-matched donors in a developing country and our findings suggest that the high percentage of patients with a low HCT-CI scores, contrary to international reports, could be explained by different comorbidities and demographics, but mainly due to stricter filters applied to HSCT candidates and consequently, a potential selection bias caused by limited resources.

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The under-representation of women in blood donation can lead to blood shortages. We aimed to determine the factors, which encourage or impede blood donations in women. The findings can help us in designing effective recruitment strategies that could encourage women to donate blood to cover the patients’ needs. This cross-sectional study was conducted in Shiraz, Iran, from the 1st of January 2017 to the 1st of August 2017 on women aged 18–60 years old. The demographic characteristics of the participants, the reasons that motivate blood donors and the factors that discourage non-donors were surveyed. Reasons for lapses in donors were also mentioned. The most frequent reasons for blood donation were altruistic causes (94.4%), moral and personal obligations (89.1%), the feeling of responsibility (82.7%), and awareness of the positive effects on their health (77.4%). The most common deterrents in non-donors were fear of developing anemia (68.4%) or weakness and dizziness (66%), unsuitability due to certain medical conditions (62.4%), and lack of a situation in which they were asked to donate blood (61.8%). Although altruistic reasons are the chief motivating factors for women to donate blood, the overall female contribution in blood donation remains low. Therefore, it is necessary to take measures aiming at informing women about the importance of blood donation and to reduce fears based on misinformation. Confidence in the blood donation organization must be a major consideration in future recruitment strategies to reassure women about the safety of blood donation.

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Abstract Introduction: Knowledge on the characteristics of neuropathic pain in people with sickle cell disease (SCD) may help to provide more effective treatment procedures. Objective: To describe the characteristics of neuropathic pain in patients with sickle cell disease and identify the impact on their quality of life. Method: A cross-sectional study (CAAE 57274516.8.0000.5544) was conducted at a reference center in Salvador, Bahia, Brazil. The instruments used were the Brief Pain Inventory (BPI), the Douleur Neuropatique Questionnaire (DN-4), the Anxiety and Depression Hospital scale (ADH) and the abbreviated version of the World Health Organization of Quality of Life questionnaire (WHOQOL-brief). The Mann-Whitney test was used to evaluate the association between the scores (5% alpha). Results: A total of 100 adults with SCD participated in the study, 69.7% of whom had neuropathic pain. Anxiety was present in 99% of the sample and depression, in 100%. Patients with neuropathic pain had worse scores in all domains of quality of life (p < 0.05), but no association was found with pain intensity. Conclusion: Neuropathic pain was more frequent than nociceptive pain in adults with SCD and generated worse scores in all domains of quality of life. Anxiety and depression were present in patients with both types of pain.

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ABSTRACT Objective: To investigate the effectiveness of a home-based therapeutic exercise program on lower back pain and functionality of SCD patients. Setting: A Hematology and Transfusion Medicine Center, University of Campinas (HEMOCENTRO-UNICAMP). Methods: This was a prospective study, with a three-month follow-up of SCD patients with lower back pain. The lumbar spine functionality was evaluated by questionnaires, trunk flexion and extension analyses by fiber-optic-electrogoniometry and measurements of muscle strength of trunk flexor and extensors. The Intervention Group (IG) comprised 18 volunteers, median age 44y (28–58) and the control group (CG) comprised 15 volunteers, median age 42y (19–58), who did not perform exercises. The protocol consisted of daily home-based exercises with two evaluations: at the beginning and end of a three-month program. In order to compare the groups at baseline, the Fishers´ exact test and Mann–Whitney test were used for categorical and numeric variables, respectively. The Wilcoxon test was used for related samples comparing numeric measures of each group over time with a 5% (p < 0.05) significance level. Results: After the intervention, patients demonstrated a significant improvement, according to the Visual-Analog-Scale (VAS; p = 0.01), Rolland Morris Disability questionnaire (RMDQ; p < 0.01) and trunk flexion and extension muscle strength (p < 0.01). No significant differences were found for the Start-Back-Screening-Tool-Brazil (SBST) and in measures of trunk flexion and extension range-of-motion (RoM). Conclusion: Results suggest that daily home-based exercises for a three-month period ameliorate pain and improve disability related to lower back pain and muscle strength.

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Abstract Introduction: Transfusion in cirrhotic patients remains a challenge due to the absence of evidence-based guidelines. Our study aimed to determine the indication of transfusion and the associated transfusion thresholds in cirrhotic patients. Methods: This retrospective observational study was conducted in the Department of Transfusion Medicine at a tertiary care liver center from October 2018 to March 2019. The blood bank and patient records of cirrhotic patients admitted during the study period were retrieved and analyzed to determine the current transfusion practice. Results: A total of 992 cirrhotic patients were included in the study. Blood components were transfused to 402 (40.5%) patients. Sixty-nine (17.2%) patients were transfused to control/treat active bleeding, while 333 (82.8%) were transfused prophylactically. Packed red blood cells (65.4%) was the most commonly transfused blood component, followed by fresh frozen plasma (35.6%), among patients receiving transfusions (therapeutic & prophylactic). The mean pre-transfusion thresholds for: (i) packed red blood cell transfusion: hemoglobin less than 7 g/dL; (ii) fresh frozen plasma transfusion: international normalized ratio over 2.6; (iii) platelet concentrate transfusion: platelet count less than 40,700/µL, and; (iv) cryoprecipitate transfusion: fibrinogen less than 110 mg/dL. The average length of stay of the study population was 5 days (3–9. Conclusion: To conclude, 40.5% of our hospitalized cirrhotic patients were transfused, with the majority of the transfusions being prophylactic (82.8%). Separate guidelines are required for this patient population, as these patients have an altered hemostasis which responds differently to the transfusion of blood components.

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ABSTRACT Background: Transfusion of ABO-compatible non-identical platelets (PTLs), fresh plasma (FP) and red blood cells (RBCs) has been associated with increased morbidity and mortality of recipients. Trauma victims are frequently exposed to ABO non-identical products, given the need for emergency transfusions. Our goal was to evaluate the impact of the transfusion of ABO non-identical blood products on the severity and all-cause 30-day mortality of trauma patients. Methods: This was a retrospective single-center cohort, which included trauma patients who received emergency transfusions in the first 24 h of hospitalization. Patients were divided in two groups according to the use of <3 or ≥3 ABO non-identical blood products. The patient severity, measured by the Acute Physiology and Chronic Health Evaluation (APACHEII) score at ICU admission, and the 30-day mortality were compared between groups. Results: Two hundred and sixteen trauma patients were enrolled. Of these, 21.3% received ≥3 ABO non-identical blood products (RBCs, PLTs and FP or cryoprecipitate). The transfusion of ≥3 ABO non-identical blood products in the first 24 h of hospitalization was independently associated with a higher APACHEII score at ICU admission (OR = 3.28 and CI95% = 1.48–7.16). Transfusion of at least one unit of ABO non-identical PTLs was also associated with severity (OR = 10.89 and CI95% = 3.38–38.49). Transfusion of ABO non-identical blood products was not associated with a higher 30-day mortality in the studied cohort. Conclusion: The transfusion of ABO non-identical blood products and, especially, of ABO non-identical PLTs may be associated with the greater severity of trauma patients at ICU admission. The transfusion of ABO non-identical blood products in the trauma setting is not without risks.

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ABSTRACT Background: Multiple myeloma is a disease of the elderly. However, 40% of patients are diagnosed before 65 years old. Outcomes regarding age as a prognostic factor in MM are heterogeneous. Method: We retrospectively analyzed clinical characteristics, response to treatment and survival of 282 patients with active newly-diagnosed multiple myeloma, comparing results between patients younger and older than 65 years. Main results: The frequency of multiple myeloma in those younger than 66 years was 53.2%. Younger patients presented with a more aggressive disease, more advanced Durie-Salmon stage (85.3% vs 73.5%; p = 0.013), extramedullary disease (12.7% vs 0%; p < 0.001), osteolytic lesions (78.7% vs 57.6%; p < 0.001) and bone plasmacytoma (25.3% vs 11.4%; p = 0.003). In spite of this, the overall response rate was similar between groups (80.6% vs 81.4%; p = 0.866). The overall survival was significantly longer in young patients (median, 65 months vs 41 months; p = 0.001) and higher in those who received autologous hematopoietic stem cell transplantation. The main cause of death was disease progression in both groups. Multivariable analysis revealed that creatinine ≥2 mg/dl, extramedullary disease, ≤very good partial remission and non-autologous hematopoietic stem cell transplantation are independent risk factors for shorter survival. Conclusion: Although multiple myeloma patients younger than 66 years of age have an aggressive presentation, this did not translate into an inferior overall survival, particularly in those undergoing autologous hematopoietic stem cell transplantation.

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Abstract Introduction: Graft-versus-host disease (GVHD) is a serious complication in allogeneic transplantation. The first-line treatment is high doses of corticosteroids. In the absence of response to corticosteroids, several immunosuppressive drugs can be used, but they entail an elevated risk of severe infections. Added to this, there are patients who do not improve on any immunosuppressive treatment, with subsequent deteriorated quality of life and high mortality. Ruxolitinib has been shown to induce responses in refractory patients. In this study we have presented our real-life experience. Methods: A retrospective analysis was performed on patients with severe GVHD refractory to corticosteroids. Demographic, previous treatment, response and mortality data were collected. Results: Since 2014, seventeen patients with GVHD were treated with ruxolitinib due to refractoriness to corticosteroids and immunosuppressants and a few to extracorporeal photopheresis, 8 with acute GVHD (1 pulmonary, 4 cutaneous grade IV and 3 digestive grade IV) and 9 with chronic GHVD (5 cutaneous sclerodermiform, 2 pulmonary and 1 multisystemic). The overall response to ruxolitinib treatment for acute GVHD was 80%, 40% with partial response and 40% with complete remission. Global response in chronic GVHD was 79%. The GVHD mortality was only seen in acute disease and was 40%. Causes of mortality in those patients were severe viral pneumonia, post-transplantation hemophagocytic syndrome and meningeal GVHD refractory to ruxolitinib. Conclusions: In our series, the use of ruxolitinib as a rescue strategy in acute or chronic GVHD was satisfactory. Ruxolitinib treatment in patients with a very poor prognosis showed encouraging results. However, the GVHD mortality remains high in refractory patients, showing that better therapeutic strategies are needed.

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Abstract Introduction: Little attention is given to thrombosis associated with pediatric acute promyelocytic leukemia (APL). This study describes the thrombotic and hemorrhagic manifestations of APL in pediatric patients and evaluates their hemostasis, based on coagulation tests. Methods: Inclusion criteria were age 0–18 years and APL diagnosis between April 2005 and November 2017. Patients who had received blood transfusion prior to coagulation tests were excluded. Baseline coagulation tests, hematologic counts, and hemorrhagic/thrombotic manifestations were evaluated. Results: Median age was 10.7 years (1–15 years). The initial coagulation tests revealed a median Hgb of 8.3 g/dL (4.7–12.9 g/dL), median leucocyte count of 10.9 × 109/L (1.1–95.8 × 109/L), median platelet count of 31.8 × 109/L (2.0–109.0 × 109/L), median activated partial thromboplastin time (aPTT) of 31.7 s (23.0–50.4 s), median aPTT ratio of 1.0 (0.78–1.6), median thromboplastin time (PT) of 17.5 s (13.8–27.7 s), median PT activity of 62% (25–95 %), and median fibrinogen of 157.7 mg/dL (60.0–281.0 mg/dL). Three patients (13%) had thrombosis. At diagnosis, 21 patients (91.3%) had bruising, one patient (4.3%) had splenic vein and artery thrombosis and one patient (4.3%) presented without thrombohemorrhagic manifestations. During treatment, two patients (8.6%) had thrombosis. Conclusion: Knowledge of thrombosis in pediatric APL is important to determine its risk factors and the best way to treat and prevent this complication.

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Hematopoietic stem cell transplantation (HSCT) is a treatment that requires long periods of hospitalization. The mobility restrictions result in physical, functional and psychological impairments. Physical exercise is a therapy that can restore physical and functional capacities; however, it is necessary to understand the effects of its practice in post-HSCT individuals. The purpose of this systematic review (SR) was to assess the impact of physical exercise in children and adolescents undergoing HSCT. The SR was conducted following the PRISMA guidelines through search in the electronic databases Embase, Lilacs, PEDro, PubMed and SCOPUS, without limitation of dates and languages. Randomized or non-randomized clinical trials with children and adolescents who underwent HSCT, aged between 3 to 19 years old, who participated in a regular physical activity program, were assessed. After removing duplicates and selecting studies according to the eligibility criteria, seven parallel studies incorporating hospitalized and discharged participants undertaking aerobic and strengthening exercises were included in this study. The main outcomes analyzed were exercise capacity, quality-of-life, body composition and freedom. Five studies comprised the meta-analysis regarding the effects of the distance walked in the 6-min walk test and quality-of-life. Physical exercise is considered to be safe, feasible and efficacious to prevent the decline of the quality-of-life in children and adolescents undergoing HCST, as well as a considerable improvement in physical capacity.

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Introduction and objective: Sickle cell anemia (SCA) is a genetic condition that alters the conformation of deoxygenated red blood cells, which results in their stiffening and the occurrence of vaso-occlusive crises, endothelium damage, organ dysfunction and systemic complications. Additionally, SCA limits the participation of individuals in physical and social activities. As we consider that physical exercise promotes the recovery of functional capacity and cardiorespiratory conditioning, we aim to verify the patterns of prescription, the effects and safety of exercise for individuals with SCA. Methodology: We systematically reviewed the published literature focusing on clinical trials that correlated physical exercise with SCA patients and cross-sectional studies that applied the stress test. The data research was based on the PRISMA recommendations and the following databases were used: Medline by PubMed, Cochrane, PEDro, Scielo. Results: Six studies which were based on the evaluation of 212 patients aged between 13 and 40 years, were selected from 122 identified studies. Those studies associated the individual effort tolerance improvement, its inflammatory profile adjustment and the absence of alteration in the autonomic nervous system activity to physical exercise or stress test. Conclusion: Low-to-moderate intensity physical exercise increased the SCA individual tolerance without causing vaso-occlusive crises, nor changes in the hemorheological and inflammatory profiles.

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Abstract Introduction: The minimal residual disease (MRD) status plays a crucial role in the treatment of acute lymphoblastic leukemia (ALL) and is currently used in most therapeutic protocols to guide the appropriate therapeutic decision. Therefore, it is imperative that laboratories offer accurate and reliable results through well standardized technical processes by establishing rigorous operating procedures. Method: Our goal is to propose a monoclonal antibody (MoAb) panel for MRD detection in ALL and provide recommendations intended for flow cytometry laboratories that work on 4-color flow cytometry platforms. Results and conclusion: The document includes pre-analytical and analytical procedures, quality control assurance, technical procedures, as well as the information that needs to be included in the reports for clinicians.

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Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and lifethreatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. Paroxysmal nocturnal hemoglobinuria results from the expansion of a clone of hematopoietic cells that due to an inactivating mutation of the X-linked gene PIG-A are deficient in glycosylphosphatidylinositol-linked proteins. Early diagnosis, using flow cytometry performed on peripheral blood, the gold standard test to confirm the diagnosis of paroxysmal nocturnal hemoglobinuria, is essential for improved patient management and prognosis. The traditional therapy for paroxysmal nocturnal hemoglobinuria includes blood transfusion, anti-thrombosis prophylaxis or allogeneic bone marrow transplantation. The treatment that has recently become available is the complement blockade by the anti-C5 monoclonal antibody eculizumab. In this consensus, we are aiming to review the diagnosis and treatment of the paroxysmal nocturnal hemoglobinuria patients, as well as the early recognition of its systemic complications. These procedures express the opinions of experts and have been based on the best available evidence and international guidelines, with the purpose of increasing benefits and reducing harm to patients.

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Myelodysplastic syndromes (MDS) are a heterogeneous group of hematological malignancies characterized by dysplasias, ineffective hematopoiesis and risk of acute myeloid leukemia transformation. Approximately 90% of MDS patients present mutations in genes involved in various cell signaling pathways. Specialized DNA polymerases, such as POLN, POLI, POLK, POLQ, POLH, POLL and REV3L, insert a nucleotide opposite replication–blocking DNA lesions in an error-prone manner and, in this way, sometimes can actively promote the generation of mutation. For the best of our knowledge, has not been described the mutations of these genes in MDS. DNA target sequencing CDS regions of the REV3L gene was performed in a 58-year-old man diagnosed as High Risk Myelodysplastic Syndrome. The patient presented very low hemoglobin, increased number of blasts, karyotype:47,XY,+8[6]/47,XY,del(7)(q32),+8[7], no response to hypomethylating therapy (decitabine), all markers of poor prognosis. Target sequencing identified a mutation c.9253-6T>C REV3L (Substitution - intronic) with VAF (variant allele frequency) = 16% considered pathogenic according to Functional Analysis through. Hidden Markov Models (FATHMM). This is the first evidence of REV3L mutation in MDS and, of utmost importance, associated with poor prognosis.