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ABSTRACT During the COVID-19 pandemic, special attention has been addressed in cancer care to mitigate the impact on the patient’s prognosis. We addressed our preparation to face COVID-19 pandemic in a Hematological and Stem Cell Transplant Unit in Brazil during the first two months of COVID-19 pandemic and described COVID-19 cases in patients and health care workers (HCW). Modifications in daily routines included a separation of area and professionals, SARS-CoV-2 screening protocols, and others. A total of 47 patients and 54 HCW were tested for COVID-19, by PCR-SARS-CoV-2. We report 11 cases of COVID-19 in hematological patients (including 2 post stem cell transplant) and 28 cases in HCW. Hematological cases were most severe or moderate and presented with several poor risk factors. Among HCW, COVID-19 were mostly mild, and all recovered without hospitalization. A cluster was observed among HCW. Despite a decrease in the number of procedures, the Transplant Program performed 8 autologous and 4 allogeneic SCT during the period, and 49 onco-hematological patients were admitted to continuing their treatments. Although we observed a high frequency of COVID-19 among patients and HCW, showing that SARS-CoV-2 is disseminated in Brazil, hematological patients were safely treated during pandemic times.

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ABSTRACT Hemostatic abnormalities and thrombotic risk associated with coronavirus disease 2019 (COVID-19) are among the most discussed topics in the management of this disease. The aim of this position paper is to provide the opinion of Brazilian experts on the thromboprophylaxis and management of thrombotic events in patients with suspected COVID-19, in the sphere of healthcare in Brazil. To do so, the Brazilian Society of Thrombosis and Hemostasis (BSTH) and the Thrombosis and Hemostasis Committee of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) have constituted a panel of experts to carefully review and discuss the available evidence about this topic. The data discussed in this document was reviewed by May 9, 2020. Recommendations and suggestions reflect the opinion of the panel and should be reviewed periodically as new evidence emerges.

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ABSTRACT Background and objective T-cell acute lymphoblastic leukemia (T-ALL) in children represents a high-risk disease. There is a lack of studies assessing the outcome of T-ALL in Hispanic populations, in which it is a rare malignancy. We report the characteristics and results of treatment for childhood T-cell ALL in children over 14 years at a Latin American reference center. Material and methods From January 2005 to December 2018, there occurred the analysis of twenty patients ≤ 16 years of age from a low-income open population diagnosed at a university hospital in Northeast Mexico. Clinical and laboratory characteristics, treatment regimens and outcomes were assessed by scrutinizing clinical records and electronic databases. Diagnosis was confirmed by flow cytometry, including positivity for CD-2, 5, 7 and surface/cytoplasmic CD3. Survival rates were assessed by the Kaplan-Meier method. Results There was a male preponderance (70 %), with a 2.3 male-to-female ratio (p = .074), the median age being 9.5 years. Leucocytes at diagnosis were ≥ 50 × 109/L in 13 (65 %) children, with CNS infiltration in 6 (30 %) and organomegaly in 10 (50 %). The five-year overall survival (OS) was 44.3 % (95 % CI 41.96–46.62), significantly lower in girls, at 20.8 % (95 % CI 17.32–24.51) vs. 53.1 % (95 % CI 50.30–55.82), (p = .035) in boys; there was no sex difference in the event-free survival (EFS) (p = .215). The survival was significantly higher after 2010 (p = .034). Conclusion The T-cell ALL was more frequent in boys, had a higher mortality in girls and the survival has increased over the last decade with improved chemotherapy and supportive care.

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ABSTRACT Background: Blood transfusion is a life-saving procedure, but may cause adverse transfusion reactions (TRs). The reporting of TRs is often missed due to various reasons. The aim of this study was to determine the incidence of unreported acute TRs through active surveillance and to compare it with the incidence of passively reported TRs. Methods: This prospective observational study was done over a period of four months at a tertiary care hospital. A total of 500 consecutive transfusion episodes (TEs) in patients who had received blood component transfusions in the intensive care units were included in the study. Comprehensive data were collected from the blood bank records, patient records and through interviews with the attending clinical staff. The TEs were defined as all blood components issued to a single patient in 24 h. Results: The overall incidence of TRs was 1.8 % (9 cases), with 0.4 % (2 cases) being reported passively, while 1.4 % (7 cases) were identified during active surveillance. The transfusion-associated cardiac overload (TACO) had the highest incidence of 1.2 % (6 cases) in active surveillance. A single case of acute hemolytic transfusion reaction was also observed during active surveillance. The passively reported TRs were one allergic reaction and one febrile non-hemolytic transfusion reaction. Conclusion: Active surveillance of TRs provided an insight into the true incidence of TRs, which is higher when compared with the passively reported TRs. The TACO was found to have the highest incidence and not a single case was reported. There is a need to improve awareness regarding TR reporting.

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ABSTRACT Objective: To develop a self-reporting questionnaire, namely the Blood Donor Satisfaction Questionnaire (BDSQ) in Brazilian Portuguese, to assess the blood donor satisfaction with the service provided by a blood bank. Method: This study was carried out in three stages: 1) a preliminary version of the instrument was proposed, based on a revision of a previous questionnaire and consideration of the focus groups blood donor perceptions; 2) a content validation to assess the relevance of the items, clarity, domain structure and comprehensiveness of the satisfaction measurement, and; 3) a pre-test of the instrument using a sample of blood donors. All the stages of the research were performed at the Regional Hemotherapy Center in Franca, São Paulo, Brazil. Results: The 25-item BDSQ being proposed has an additional question on overall satisfaction. Responses to items are presented on a five-point scale, including a neutral answer. The BDSQ has three domains: accessibility and convenience, technical aspects, and interpersonal aspects. Conclusion: The application of the BDSQ has shown that it can be a useful tool in generating information for managers on specific aspects of blood bank services which need to be improved, monitored or maintained, aiming to provide a service which meets the blood donor expectations and makes him or her feel satisfied with the care received.

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ABSTRACT Objective: The aims of this study were to identify the main characteristics regarding the shape and size of the craniofacial region in patients with sickle cell anemia (SCA) and sickle cell trait (SCT) and in unaffected patients using geometric morphometrics and to check the efficiency of this method. Material and Methods: A cross-sectional analytical study of 45 patients (15 in each group) was performed. Lateral radiographs of the skull were used for the analysis. Seventeen landmarks and semilandmarks were placed for the measurements. The Pocrustes analysis of variance (ANOVA), regression analysis, multivariate analysis of variance, canonical variate analysis, Mahalanobis and Procrustes distances and unweighted pair group method with arithmetic mean (UPGMA) clustering were performed. Allometric effects and sex characteristics were not statistically significant (p > 0.05). Results: There were, however, significant differences (p < 0.05) in craniofacial shape among SCA, SCT and unaffected individuals. Those with SCA showed variations in the shape of the external auditory meatus and at the base of the occipital bone, in addition to the mandibular setback and upper incisor inclination, with a tendency towards prognathism. The individuals with SCT exhibited a similar craniofacial shape to those with SCA, but with slighter variations. Moreover, those with SCT were statistically closer in resemblance to unaffected individuals, given that SCT is not regarded as a disease. Conclusion: This demonstrates the efficiency of geometric morphometrics in the categorization of the assessed groups.

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ABSTRACT Background: The minimal residual disease (MRD) is the most important prognostic factor for acute lymphoblastic leukemia (ALL) in children. This study aimed to investigate the influence of detecting the MRD by the multiparametric flow cytometry (MFC) at day 15 (D15) of the induction on the analysis of the risk group classifications of the different childhood ALL treatment protocols used in a referral hospital in southern Brazil. Method: We retrospectively reviewed the medical records of patients with B-cell ALL, aged 1 to 18 years, treated at a hospital from January 2013 to April 2017. Main results: Seventy-five patients were analyzed. Regarding the MRD by the MFC at D15, the analyses showed statistical significance when the MRD was grouped into three categories, < 0.1%, 0.1–10%, and > 10%, with the following distribution: 30.7%, 52.0%, and 17.3%, respectively. There was a significant association between D15 MRD-MFC < 0.1% and the likelihood of dying or relapsing and between D15 MRD-MFC > 10% and the likelihood of dying or relapsing. The cumulative hazard ratio for the relapse of patients with D15 MRD-MFC < 0.1%, 0.1–10%, and > 10% was 19.2%, 59.8%, and 80.1%, respectively. Conclusion: Our analysis suggests D15 MRD-MFC < 0.1% as a cut-off point for patients with more favorable outcomes and that the MRD at D15 in risk classifications is particularly useful for the stratification of patients with a more favorable prognosis.

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ABSTRACT Hemophilia is an X-linked recessive genetic disorder which affects approximately 400,000 people globally. Differing healthcare reimbursement systems, budgetary constraints and geographical and cultural factors make it difficult for any country to fully deliver ideal care. Although developed countries have sufficient treatment products available, they are burdened by the higher expectation of outcomes, coupled with insufficient supportive care to monitor adherence and outcomes and to implement regular follow-up. In contrast, developing regions may not have ready access to factor replacement, but have developed excellent physiotherapy and rehabilitation programs. Although there are multiple studies that have attempted to assess country-specific variations in hemophilia care, very few compare hemophilia care between economically unequal countries and the challenges in achieving optimal hemophilia care. This literature review tries to bridge this gap and throws light on the country-specific differences in epidemiology, standard of hemophilia care and challenges faced in Canada and China. Data sources resulted in 20 studies (11 from Canada and 9 from China), which were reviewed. In a developed country, the main advantages are: the early treatment of bleeding episodes and the presence of a specialized interdisciplinary and comprehensive treatment concept. This is not the case in most developing countries, where the government does not have the resources to buy the necessary quantities of coagulation factors in the face of more urgent health priorities and hardly a few patients can afford to pay for their own treatment, even the on-demand home therapy.

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ABSTRACT Background: We evaluated different technological approaches and anti-D clones to propose the most appropriate serologic strategy in detecting the largest numbers of D variants in blood donors. Methods: We selected 101 samples from Brazilian blood donors with different expressions of D in our donor routine. The tests were performed in immediate spin (IS) with eleven commercially available anti-D reagents in a tube and microplate. The D confirmatory tests for the presence of weak D included the indirect antiglobulin test (IAT) in a tube, gel and solid-phase red blood cell adherence (SPRCA). All DNA samples were extracted from peripheral blood and the D variants were classified using different molecular assays. Results: The RHD variants identified by molecular analysis included weak D types (1, 2, 3, 11 and 38) and partial Ds (DAR1.2, DAR1, DAR3.1, DAU0, DAU2, DAU4, DAU5, DAU6, DMH and DVII). The monoclonal-monoclonal blend RUM-1/MS26 was the best anti-D reagent used in detecting the D antigen in the IS phase in a tube, reacting with 83.2% of the D variants, while the anti-D blend D175 + 415 was the best monoclonal antibody (MoAb) used in a microplate to minimize the need for an IAT, reacting with 83.2% of the D variants. The D confirmatory tests using SPRCA showed a reactivity (3 - 4+) with 100% of the D variant samples tested. Conclusion: Our results show that, even using sensitive methods and MoAbs to ensure the accurate assignment of the D antigen, at least 17% of our donor samples need a confirmatory D test in order to avoid alloimmunization in D-negative patients.