Resumo em Inglês:

ABSTRACT Background: Hematologic neoplasms are associated with mutations in hematopoietic cells and chromosomal abnormalities. During aging, about 2–3% of the elderly have chromosomal abnormalities arising from clonal mosaicism, the immune system is impaired and the bone marrow loses its ability to replace blood cells. Objective: To describe the epidemiological and cytogenetic profile of hematological malignancies, highlighting the frequency of chromosomal alterations in these neoplasms associated with aging. Method: A retrospective cross-sectional study with analysis of karyotype exams results was performed in the Cytogenetic Laboratory of thee Blood Center of the Faculdade de Medicina de Marilia (FAMEMA) between 1998 and 2016. Blood samples from child and adult patients with different hematological malignancies treated in the Onco-hematology Outpatient Clinics of the local blood center and hospitals, and external clinics were tested. Results: Karyotype exam results of 746 patients with a mean age of 54.7 years (±23.1) were analyzed. The elderly had the highest frequency of hematological malignancies (50.9%), followed by adults (38.3%) and young people (10.7%); elderly women had the highest percentage (55.0%). Normal karyotypes (46,XX/46,XY) were more common (61.8%) compared to abnormal karyotypes, especially among the elderly (56.4%). Myeloproliferative neoplasms were an exception with 67.4% of abnormal karyotypes. Conclusion: There is a higher frequency of hematological malignancies among the elderly. It is possible to conclude that failures in genomic mechanisms and hematopoiesis with aging lead to the formation of cells with the chromosomal alterations found in hematological malignancies.

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ABSTRACT Objective: To identify barriers to the self-care practice of young people with sickle cell disease. Method: This qualitative study was conducted with 17 individuals with sickle cell disease aged between 13 and 24 years in Belo Horizonte, MG, Brazil in March and April 2017. An interview investigated the barriers to self-care practice and the feelings associated with sickle cell disease. Data were transcribed and analyzed according to Bardin's perspective using the following steps: (1) pre-analysis, (2) exploration of the material, and (3) treatment of the results (inference and interpretation). Results: Five thematic categories emerged: (1) feelings: anger, sadness, and fear; (2) bullying and stigmatization: challenges regarding walking, speaking, or behaving, as well as patient labels; (3) cognitive factors: doubts related to medication, hydration, heredity and maternity; (4) medication compliance: fear of the side effects suffered and anger triggered by the obligation to use the medication; (5) family issues: complaints of not earning the mothers’ trust to live independently. Conclusion: The barriers to self-care in young people with sickle cell disease indicate difficulties related to emotional, behavioral, and environmental aspects. Understanding these factors will favor a better adaptation of youths to the context of sickle cell disease.

Resumo em Inglês:

ABSTRACT Background: Due to laboratory logistic issues, our center has traditionally scheduled peripheral blood stem cell harvests based on timing from the start of mobilization. This has proved to be useful in some cases, but also resulted in many fruitless harvests due to poor mobilization. In order to improve the efficiency of collections and compare the effectiveness of peripheral blood CD34+ cells as a predictor with data from other reports, this study analyzed the implementation of this routine. Methods: Peripheral blood and leukapheresis samples were quantified by flow cytometry and the association between these parameters was assessed. Results: Sixty-six consecutive leukapheresis samples were collected from 34 patients after the collection of peripheral blood samples for CD34+ quantification. A moderate positive correlation was observed between peripheral blood CD34+ cell count and total CD34+ cell count/kg (r = 0.596; p-value < 0.001). A multivariable regression model also confirmed this association and allowed the estimation that for every increase in five CD34+ cells/µL in the peripheral blood, a mean increase of 0.38 × 106 CD34+ cells/kg could be predicted. Demographic characteristics, baseline comorbidities and mobilization regimen did not influence final CD34+ cell count in this sample. Conclusions: As observed in other centers, quantification of peripheral blood CD34+ progenitor cells is a strong predictor of effectiveness to guide stem cell harvesting. Due to the results of this study, a modification in the peripheral blood stem cell harvesting logistics was implemented at our center in order to incorporate this routine.

Resumo em Inglês:

ABSTRACT Background: Refractory or unexplained iron deficiency anemia accounts for about 15% of all cases. The endoscopic gastrointestinal workup sometimes fails to establish the cause of iron deficiency anemia and a considerable proportion of patients regardless of risk category fail to respond to oral iron supplementation. The aim of the present study was to assess the etiological role of Helicobacter pylori infection in adult Egyptian patients with unexplained or refractory iron deficiency anemia. Methods: A case controlled study was composed of 104 iron deficiency anemia cases and 70 age- and gender-matched healthy controls. Patients were diagnosed with iron deficiency anemia according to hemoglobin, mean corpuscular volume, serum ferritin, and transferrin saturation. Upper and lower endoscopies were performed and active H. pylori infection was investigated by testing for the H. pylori antigen in stool specimens. Hematological response to H. pylori treatment with triple therapy together with iron therapy (n = 32) or only iron therapy (n = 32) were assessed in patients with H. pylori infection. Results: H. pylori infection was more prevalent in patients with unexplained or refractory iron deficiency anemia (61.5%). Of the different hematological parameters investigated, there was a significant correlation only between H. pylori infection and mean corpuscular volume (p-value 0.046). Moreover, there was a significant correlation between receiving triple therapy together with iron supplementation and improvements in the hematological parameters [hemoglobin (p-value < 0.001), mean corpuscular volume (p-value < 0.001), iron (p-value < 0.001) and serum ferritin (p-value < 0.001)] compared to receiving iron supplementation alone. Conclusions: Failing to test for H. pylori infection could lead to a failure to identify a treatable cause of anemia and could lead to additional and potentially unnecessary investigations. Furthermore, treatment of H. pylori infection together with iron supplementation gives a more rapid and satisfactory response.

Resumo em Inglês:

ABSTRACT Background: The Children's Hospital Oakland Hip Evaluation Scale is a disease-specific tool for the clinical and functional assessment of the hip in sickle cell disease. Objectives: To translate the tool into Brazilian Portuguese and evaluate the interobserver and test–retest reliability. Methods: Eighteen patients diagnosed with sickle cell disease and a mean age of 49 ± 11.9 years participated in the study. The scale was applied by two evaluators who did not speak to each other regarding their understanding of the tool and who had no prior training. Interobserver and test–retest reliability of individual items and of the total score were evaluated using the intraclass correlation coefficient and the Bland–Altman method. Results: When the overall score for each hip was considered, the test–retest intraclass correlation coefficient score for the right hip was 0.95 (0.89–0.98) and for the left hip it was 0.96 (0.91–0.98). Considering all assignments (total score), the score was 0.96 (0.90–0.98). The test–retest intraclass correlation coefficient varied from 0.76 to 1 for 18 of the 27 items (excellent) and from 0.53 to 0.75 for nine items (moderate). When the overall score for each hip was considered, the interobserver intraclass correlation coefficient for both hips was 0.94 (0.86–0.98). Considering all assignments, the total score was 0.94 (0.86–0.98). The interobserver intraclass correlation coefficient varied from 0.48 to 0.75 for 18 out of 27 items (moderate) and varied from 0.77 to 1 for the remaining nine items (excellent). Conclusion: The results demonstrate that the Brazilian version of the Children's Hospital Oakland Hip Evaluation Scale presented adequate interobserver and test–retest reliability and that the version can be used to evaluate clinical function in sickle cell disease patients, producing consistent, standardized and reproducible results.

Resumo em Inglês:

ABSTRACT Background: Peripheral blood stem cell concentrations are traditionally adjusted to 20–40 × 106 leukocytes/mL prior to freezing. This low cell concentration at cryopreservation implies larger volumes with more dimethyl sulfoxide being used, and higher cost and toxicity at the time of transplant. Higher cell concentrations have been reported but this is not widely accepted. Moreover, the influence of cell concentration on engraftment has not been well documented. Therefore, this study retrospectively analyzed the influence of peripheral blood stem cell concentration at freezing on engraftment after autologous hematopoietic stem cell transplantation. Method: Leukapheresis products were plasma-depleted and cryopreserved with 5% dimethyl sulfoxide, 6% hydroxyethylamide solution and 4% albumin in a −80 °C freezer. Individual patient data from hospital records were reviewed. Results: Fifty consecutive patients with oncological diseases underwent 88 leukaphereses. Median age was six years (range: 1–32 years) and median weight was 19 kg (range: 8–94 kg). Median leukocyte concentration was 109 × 106/mL at collection and 359 × 106 (range: 58–676 × 106) at freezing with 78% viability (range: 53–95%); leukocyte recovery after thawing was 95% (range: 70–100%). In multivariate analysis, cell concentration (p-value = 0.001) had a negative impact on engraftment. Patients infused with bags frozen with <200 × 106 leukocytes/mL engrafted after a median of nine days (range: 8–12 days), 200–400 × 106 leukocytes/mL after 11 days (range: 9–20 days); 400–600 × 106 leukocytes/mL after 12 days (range: 8–19 days) and with cell concentrations >600 × 106 leukocytes/mL, engraftment was after 14 days (range: 13–22 days). Conclusion: In patients with adequate CD34 cell collections, total leukocyte concentrations of 282 × 106/mL, freezing with 5% dimethyl sulfoxide and 6% hydroxyethylamide solution without a controlled-rate freezer, and storing cells at −80 ºC yielded excellent engraftment. Further increases in cell concentration may delay engraftment, without affecting safety.

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ABSTRACT Background: Oral manifestations may be the first signs of hematologic diseases, and may occur due to the disease itself or to treatment. Objective: To evaluate the frequency and types of oral conditions presented by patients on a hematology ward. Methods: Data were collected by oral examinations during weekly visits to a hematology ward. Six trained dentists performed the oral assessment based on the principles of oral semiology. All patients who accepted to be examined were included in the study. Patients who were unavailable or unable to have oral examinations were excluded. Data were recorded on protocol forms and in the electronic records of the institution. A descriptive analysis was performed. Results: Seventy-nine patients were included in the analysis; 50.6% were female and the mean age was 41.49 years. The most common reasons for hospitalization were chemotherapy and complications (81%), relapse (13.9%) and pre-transplant preparation (5%). The most frequent underlying diseases were multiple myeloma (17.7%), acute myeloid leukemia (15.4%) and acute lymphocytic leukemia (11.5%). Oral conditions were found in 36 (45.6%) patients, some of whom presented more than one condition. The most common oral conditions were dry lips (12.6%), mucositis (10.1%), petechiae (8.9%) and candidiasis (7.6%). Of the detected oral conditions, 56.9% were related to the underlying disease or chemotherapy and 20.2% were not related to the disease. Conclusion: This study shows the types and frequency of oral conditions observed in hematological inpatients. Awareness of these conditions is important for prevention and planning the care of patients with hematological diseases.

Resumo em Inglês:

ABSTRACT Objective: To investigate, in a large prospective multicenter study, whether 2-[18F]-fluoro-2-deoxy-D-glucose-positron emission tomography is sufficiently accurate to identify clinically important bone marrow involvement by Hodgkin's lymphoma to replace routine bone marrow biopsy in a developing tropical country. Methods: Patients newly diagnosed with Hodgkin's lymphoma were recruited from six cancer centers in Brazil. All were staged by the results of positron emission tomography/computed tomography that were centrally reviewed and by iliac crest bone marrow biopsy. Patients were classified as having marrow disease if they had lymphoma identified by marrow biopsy histology or had focal 2-[18F]-fluoro-2-deoxy-D-glucose marrow uptake that resolved following chemotherapy. Results: A total of 246 participants were recruited from six different centers and 62 (25.2%) were judged to have Hodgkin's lymphoma in the bone marrow. Positron emission tomography and biopsies were concordant in 206 patients (83%). Positron emission tomography correctly identified marrow disease in 59/62 patients (95.1%) and marrow biopsy in 25/62 patients (40.3%). In 22/62 (35.4%) patients, the two techniques were concordant in the diagnosis of marrow involvement. Of the forty discordant results, positron emission tomography found bone marrow involvement in 37 patients, upstaging 22 to stage IV and having an impact on therapeutic decision in nine cases given their reallocation from early to advanced stage. Three false negative positron emission tomography results were obtained with bone marrow biopsy giving positive findings. All three cases were classified as stage IV regardless of bone marrow findings implying no modification in the clinical management. The sensitivity, specificity and accuracy of positron emission tomography for detecting bone marrow disease were 95%, 100% and 98% and for bone marrow biopsy they were 40%, 100% and 84%, respectively. Conclusion: We conclude that positron emission tomography can replace marrow biopsy in Brazilian patients with Hodgkin's lymphoma without compromising clinical management.

Resumo em Inglês:

ABSTRACT Background: Zika, a disease caused by Zika virus infections, has recently emerged and caused outbreaks in many parts of the world. The clinical manifestations of Zika are usually mild, mostly presenting as an exanthematic febrile disease, but on some occasions, it might be associated with microcephaly after intrauterine infection, and Guillain-Barré Syndrome. Zika virus is primarily transmitted by mosquito bites, but other means of transmission have been described, and potential risk for blood transmission has been reported in French Polynesia and Brazil. Methods: To investigate the risk of Zika virus infection after a blood transfusion in an area of Brazil where a possible transmission by a platelet concentrate has been described. Using a mini-pool format, 1857 blood donations were evaluated by real-time reverse transcriptase polymerase chain reaction designed to detect Zika virus RNA. Results: After testing samples individually from positive mini-pools, the prevalence of Zika virus RNA was only 0.16%, a result probably associated to the low circulation of this virus in the study area. In addition, it was evident that the implementation of post-surveillance programs is important to detect Zika virus infections in blood donors, as the post-donation surveillance program detected two blood donors with the disease in this study. Conclusion: This study shows that the risk for Zika virus transmission by blood transfusion is real, even in regions with a low circulation of the disease, but the combination of the detection of Zika virus RNA by polymerase chain reaction and post-donation surveillance might reduce the risk of transmission by blood transfusions.

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ABSTRACT Based on the literature, this work aimed to discuss infection by Chagas disease among blood donors in Brazil. Studies on the prevalence of Trypanosoma cruzi infection in donors or candidates for blood donation in Brazil are important. The prevalence of infection appears to be a sensitive indicator and can be a true marker of the risk of the transmission of Chagas disease by blood transfusion. Moreover, it serves as a marker of the level of transmission of the disease in a region, as well as a tool to characterize the epidemiological profile of individuals affected by the disease. The present study is an integrative review of the literature on chagasic infection among blood donors. An evaluation of the literature identified the epidemiological profile of blood donors infected by T. cruzi, which is characterized in general as men, over 30 years old, with a low level of schooling, low income and mainly coming from rural areas.