Hematology, transfusion and cell therapy最新文献

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.

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.

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.

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.

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.

The central role of the control of apoptosis in the pathophysiology of Philadelphia chromosome-negative myeloproliferative neoplasms has recently been reinforced in genetic and pharmacological studies. The inhibitor of apoptosis protein family has eight members and plays an important role in apoptosis, with the most studied being survivin (BIRC5) and X-linked inhibitor of apoptosis (XIAP). YM155 is a small molecule with antineoplastic potential that has been described as a suppressant of survivin and XIAP. In the present study, BIRC5 expression was significantly increased in primary myelofibrosis patients compared to healthy donors. On the other hand, XIAP expression was reduced in myeloproliferative neoplasms patients. In JAK2^V617F cells, YM155 reduces cell viability and autonomous clonal growth and induces apoptosis, cell cycle arrest, and autophagy. HEL cells that show greater malignancy are more sensitive to the drug than SET2 cells. In the molecular scenario, YM155 modulates apoptosis-, cell cycle-, DNA damage- and autophagy-related genes. Protein expression analysis corroborates the observed cellular phenotype and exploratory gene expression findings. In summary, our results indicate that survivin/BIRC5 and XIAP are differently expressed in myeloproliferative neoplasms and YM155 has multiple antineoplastic effects on JAK2^V617F cells suggesting that inhibitor of apoptosis proteins may be a target for pharmacological interventions in the treatment of these diseases.