Resumo em Inglês:

Abstract Background This study aimed to describe the incidence, microbiological profile, and risk factors associated with bacteremia in pediatric patients with hematopoietic stem cell transplantation (HSCT). Methods A retrospective cohort study was performed on patients under 18 years old who underwent HSCT due to any indication, between January 2012 and January 2017. The patient data were collected from the first 100 days post-HSCT. Bacteremia was defined as the isolation of bacteria from at least one blood culture. The relation between bacteremia and associated risk factors was evaluated using univariate and multivariate analysis. Results We enrolled a total of 111 pediatric patients who received HSCT during the period of study. The total number of patients who developed at least one episode of bacteremia was 46 (41.4%), and the total number of bacteremia episodes was 62. Rectal swabs were performed in 44 patients to detect prior colonization and this showed that 25% of them were positive, the main pathogen isolated being carbapenem-resistant enterobacteriaceae. The Gram-negative bacteria cases accounted for 60% of 62 events. The main Gram-negative bacteria isolated were Klebsiella pneumoniae and Escherichia coli and the majority were resistant strains. The mortality rate on day +100 for the whole cohort was 18% (n = 20). Regarding the patients with bacteremia, the mortality rate on day +100 was 30% (n = 14), and the cause of death was related to infection in 10 of them. We did not find any statistically significant risk factor for the development of bacteremia. Conclusion Bacteremia is common after HSCT, and there is a high frequency of resistant Gram-negative infections which is related to high mortality.

Resumo em Inglês:

Abstract Introduction About 10% of sickle cell anemia patients will have ischemic stroke. Adams showed stroke incidence reduction in children receiving monthly erythrocyte transfusions by reducing transcranial Doppler (TCD) velocities. Since then, chronic transfusion is recommended as primary stroke prophylaxis. This study aims to assess the effectiveness of chronic transfusions as stroke prophylaxis. Method Retrospective study, reviewing medical records from 15 sickle cell anemia patients undergoing chronic transfusion. Data collected were age, sex, adverse reactions, stroke, hemoglobin, reticulocytes, ferritin, HbS and TCD values (baseline, after 12 and 24 months of treatment). Results The mean age was 118.67 ± 41.40 months; six patients experienced allergic reactions. No stroke was recorded. One patient had alloimmunization. There was a decrease in the HbS rate and an increase in hemoglobin values in the first 12 months. Values were maintained after 24 months, but with no improvement of data. Before treatment, the mean HbS rate was 75.18%±11.69; after 12 months, 41.63 ± 14.99 and after 24 months, 43.78 ± 10.6. Thirteen patients initiated chelation after 12 months from the beginning of chronic transfusions and ferritin decline after 24 months. Pre-transfusional TCD velocities were 204.28 ± 9.41 cm/s (right) and 198.85 ± 33.37 cm/s (left). After a 12-month treatment, these values were 158.5 ± 28.89 cm/s and 157.62 ± 34.43 cm/s, respectively, and this reduction was statistically significant (p = 0.002 right and p = 0.02 left). After 24 months, these values were 149.63 ± 26.95 cm/s (right) and 143.7 ± 32.27 cm/s (left). Conclusion Significant reduction of TCD velocity occurred after treatment with chronic transfusion in sickle cell anemia patients, leading to a normal or conditional test and reducing stroke risk in all but one patient.

Resumo em Inglês:

Abstract Objective Cytomegalovirus infection and disease are significant causes of morbidity and mortality among patients with hematopoietic stem cell transplantation. The aim of this study was to assess the frequency of cytomegalovirus infection and characterize the patients who developed the disease. Methods A retrospective cohort study was performed among adult patients, recipients of allogeneic HSTC between 2008 and 2015. Taking into account the institutional protocol of prophylaxis infections in hematopoietic stem cell transplantation, patients received either preemptive therapy or prophylaxis with valganciclovir. Infection was defined as a positive pp65 antigenemia assay or PCR higher than 500 copies/mL. Disease was defined as viremia with evidence of end organ damage. Results Seventy patients were included, the median age was 36 years old (IQR 17–62). A total of 93% of the recipients had a positive serology. The Cytomegalovirus infection occurred in 59% of the patients. Eleven patients developed disease (16%), the most frequent manifestation being colitis, followed by pneumonitis and a single case of retinitis. There were no differences between the preemptive therapy or prophylaxis groups. The mean time of onset of the disease was day 94 post-transplant. Three patients developed disease with a viral load lower than 1000 copies/mL. Conclusion The incidence of cytomegalovirus infection after transplantation at our institution is high. It was found that the disease can occur with any level of viral load and is associated with high mortality.

Resumo em Inglês:

Abstract Introduction The correct completion of the blood components request form is the starting point to ensure good transfusion care. Many guidelines have been developed to search for hemoglobin values that trigger the need for transfusion and show the importance of Patient Blood Management, a scientific evidence-based approach in processes where transfusion is a possibility, such as in elective surgeries. Objective The cross-sectional study aimed to analyze the transfusion requests at a complex orthopedic hospital over a one-year period. Method The completion quality was classified as Good, Regular, Bad or Very Bad, according to the information given by the physician. Transfusion medicine professionals analyzed the transfusion indication reported on the request form and classified it as Correct, Not based on Patient Blood Management (PBM), in accordance or not with the institutional Maximum Surgical Blood Order Schedule, Impossible to evaluate due to lack of information on the form, and Incorrect. Results The study categorized the completion quality of 2011 requests as Good (8.80%), Regular (9.30%), Bad (72.75%) and Very Bad (9.15%). Analysis of the indications revealed that 54.90% of the requests were in accordance with the current blood transfusion recommendations, and on 23.12% of the forms this field had not been filled out. Conclusion The majority of blood components (63%) requests are in tune with current blood transfusion recommendations, despite the great number of incorrectly completed forms; nevertheless, it is mandatory to reach much better appropriateness rates.

Resumo em Inglês:

Abstract Introduction Hematologists deal every day with high mortality rates of acute leukemia patients. Many times these patients need Intensive Care Unit (ICU) support and some general ICU teams believe that these patients have a much greater chance of dying than patients with other pathologies. In Brazil, data related to mortality rates and ICUs for acute leukemia patients are scarce. Methods Therefore, to assess mortality predictors in patients with acute leukemia admitted to a specialized hematological ICU, we evaluated demographics, supportive care, hospitalization time, disease status, admitting diagnosis, neutropenia, number of transfusions and Acute Physiology and Chronic Health Evaluation (APACHE)/Sepsis Related Organ Failure Assessment (SOFA) scores as possible factors associated with mortality. Data were extracted from the first admission records of 110 patients with acute leukemia admitted to the Hemocentro de Pernambuco (Hemope) ICU between 2006 and 2009. Results In this retrospective cohort study, 72/110 of the patients were men, and 64/110 were from the metropolitan area of Recife. The patients’ age median was 43.5 years (±17.9); 67.3% had acute myeloid leukemia (AML) and 32.7% had acute lymphoid leukemia. The main admitting diagnosis in the ICU was sepsis (66.7%). The mean APACHE II score was 18.3. Of the total, 65 (59%) died, and the mortality rate was independently related to longer hospitalization (p < 0.001), the increase in the APACHE II score (p < 0.038) and having received hemodialysis (p < 0.006). Neutropenia, receiving multiple transfusions and using any kind of mechanical ventilation or vasoactive drug on admission were not relevant to mortality. Factors associated with higher mortality rates were: longer hospitalization, increase in the APACHE II score, and use of hemodialysis. Conclusion With these data, to prevent organ lesions before admission to the ICU, a better strategy might be to reduce mortality for leukemia patients.

Resumo em Inglês:

Abstract Introduction Patients with benign or malignant blood disorders, who require allogeneic stem cell transplantation and lack an identical human leukocyte antigen HLA identicalHL sibling donor, could be transplanted with hematopoietic stem cells from unrelated adult or umbilical cord donors. However, in our country, both approaches are costly and time-consuming options. Methods Over the last few years, haploidentical modalities have been investigated as an alternative donor source, showing similar results to those obtained with identical HLA donors. We started using T-cell-replete haploidentical with post-transplant cyclophosphamide in 2012 and we presented our experience with patients undergoing haploidentical ransplantation compared to SIB. Results Since January 2012 to date, 91 allogeneic transplants have been performed, of which 49 were haploidentical and 42 were HLA identical. The mean age of the patients was 35 years (range: 17–62). The mean CD34/kg × 106 infused per group was 5.93 and 5.89, respectively. Time to granulocyte and platelet engraftment was 11 and 15 days, respectively, for haploidentical, and 12 and 14 days, respectively, for HLA identical (p = 0.10). The 100-day cumulative incidence of global acute GVHD was 34% for haploidentical and 29% for SIHLA identical (p = 0.9). The 2-year overall global graft-versus-host disease was 43% for haploidentical and 41% for HLA identical (p = 0.8). Overall survival, relapse, and transplant and relapse-related mortality were similar between both groups. Conclusion Our experience showed that haploidentical has similar outcomes to those obtained with HLA idential and can be performed in our country safely.

Resumo em Inglês:

Abstract Introduction Ruxolitinib has been approved for the treatment of myelofibrosis (MF). In this study, we present safety and efficacy findings from an analysis of 104 patients with intermediate- and high-risk MF in a Brazilian cohort of the JUMP study who received treatment with ruxolitinib. Methods JUMP is a single-arm, open-label, phase IIIb, expanded-access study. The primary endpoint was to evaluate the safety and tolerability (frequency, duration, and severity of adverse events [AEs]) of ruxolitinib. Results All of the 104 patients received the treatment. Median duration of exposure was 35.8 months. The most common hematologic AEs were anemia (57.7), thrombocytopenia (38.5%), neutropenia (11.5%), and leukopenia (9.6%). Second malignancies (all grades) occurred in 19.2% of patients (n = 20). Serious AEs were reported in 62.5% of patients (n = 65). The proportions of patients with ≥50% reduction from baseline in palpable spleen length at weeks 24 and 48 were 62.7% and 69.2%, respectively. The mean change from the baseline in the Functional Assessment of Cancer Therapy (FACT)-Lymphoma total score was 10.8 [15.6%] at week 4, 12.6 [14.1%] at week 24, and 12.2 [14.3%] at week 48. The mean change from the baseline for the Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue scale was 3.9 [42.8%] at week 4, 4.9 [29.9%] at week 24, and 4.7 [28%] at week 48. At week 48, the estimated progression-free survival, leukemia-free survival, and overall survival probabilities were 91%, 91% and 93%, respectively Overall, 21 deaths were observed in the present study. Conclusion Findings from this study suggest that ruxolitinib could be evaluated as a standard-of-care treatment for the MF population in need of a viable treatment option. NCT01493414

Resumo em Inglês:

Abstract Introduction Acute lymphoblastic leukemia (ALL) is the cancer with the highest incidence in childhood and adolescence, and pharmacotherapy is the primary form of treatment. Objective and methods A systematic review of the efficacy and safety of polyethylene glycol (PEG)-asparaginase in acute lymphoblastic leukemia therapy in children and adolescents was conducted to compare it with native Escherichia coli L-asparaginase. PubMed, Web of Science, Science Direct, Cochrane Library, Scopus, LILACS (Latin American and Caribbean Health Sciences Literature) and EMBASE databases were selected. The following outcomes were analyzed: complete remission of the disease, event-free survival, overall survival, anti-asparaginase antibody level, hypersensitivity reactions, asparaginase and asparagine serum levels, number of postdiagnosis events, and overall mortality. Five randomized controlled trials were included. Analysis of the quality of evidence and risk of bias was performed using the Cochrane recommendation tool and the GRADE system. Results The assessment results suggest that the level of certainty on the technology addressed is relatively weak from a methodological point of view. Evidence is insufficient to assess the effects on health outcomes because of the limited number and power of studies and important flaws in their design or conduct in classifying PEG-asparaginase as a superior drug or not, in the pharmacotherapy of ALL in children and adolescents. PEG-asparaginase can be used as a substitute for native E. coli L-asparaginase, demonstrating similar efficacy and safety. Conclusion The study may help decision-makers in the public health system to offer a more in-depth judgment on the therapeutic alternatives used to treat this neoplasm in children and adolescents.

Resumo em Inglês:

Abstract Due to the longer survival of critically ill children, venous thromboembolism is a problem which is becoming increasingly recognized in pediatric practice. In the last decades, several international studies have been published, shedding a light upon the epidemiology of this disease during childhood. These data show peculiarities in the clinical presentation and the significant morbidity and mortality. The new “epidemic of thrombosis” in pediatric hospitals points toward the urgent need for specific treatment and prevention protocols targeting this population. In Brazil, knowledge regarding this disease remains scarce. The lack of epidemiological data impacts both the clinical care and the design of specific public policies in the field. Thus, a national registry of pediatric venous thromboembolism is relevant to the proposal of an appropriate plan of action to create a qualified net of assistance. The improvement in educational initiatives related to the field of Pediatric Hemostasis is also very important. In this review, we have updated the epidemiological, clinical and therapeutic aspects of the disease, as well as the prevention strategies.

Resumo em Inglês:

Abstract Inflammation and angiogenesis are linked to the development of cancer since both can support the establishment of a tumor-prone microenvironment. The CCR5 is a major regulatory molecule involved in inflammation. The CD34 molecule is commonly described as a hematopoietic stem cell marker, and CD34+ cells are involved in the regulation of distinct physiological processes, including angiogenesis. CCR5 participates in the development of various types of cancer, and recently, a reduced CCR5 expression was associated with low CD34+ cell counts in human cord blood. A naturally occurring genetic variant of the CCR5 gene, the so-called CCR5Δ32 polymorphism, consists of a 32 base-pair deletion in the DNA, interfering in the CCR5 protein levels on the cell surface. When in homozygosis, this variant leads to a total absence of CCR5 expression on the cell surface. In heterozygous individuals, CCR5 surface levels are reduced. Based on these key findings, we hypothesize that a functional interaction can connect CCR5 and CD34 molecules (giving rise to a “CCR5-CD34 axis”). According to this, a CCR5-CD34 interaction can potentially support the development of different types of cancer. Consequently, the lack of CCR5 in association with reduced CD34+ cell counts could indicate a protective factor against the development of cancer. It is required to characterize in detail the functional relationship between CCR5 and CD34 proteins, as well as the real influence of both molecules on the susceptibility and development of cancer at population level. If our hypothesis is confirmed, the CCR5-CD34 axis may be a potential target in the development of anti-cancer therapies.