Logomarca do periódico: Hematology, Transfusion and Cell Therapy

Open-access Hematology, Transfusion and Cell Therapy

Publicação de: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular (ABHH)
Área: Ciências Da Saúde
Versão impressa ISSN: 2531-1379
Versão on-line ISSN: 2531-1387
Título anterior: Revista Brasileira de Hematologia e Hemoterapia
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Hematology, Transfusion and Cell Therapy, Volume: 47, Número: 3, Publicado: 2025

Hematology, Transfusion and Cell Therapy, Volume: 47, Número: 3, Publicado: 2025

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Documents
Original article
Long-term follow-up results of ruxolitinib as salvage therapy for chronic graft-versus-host disease Sanli, Neslihan Mandaci Karakuş, Esen

Resumo em Inglês:

Abstract Introduction Chronic graft-versus-host disease poses a significant challenge after allogeneic hematopoietic stem cell transplantation with initial treatment often relying on high-dose steroids. However, managing steroid-refractory disease remains daunting. Recent insights into the mechanisms have unveiled new treatment targets, with ruxolitinib, a selective JAK1/2 inhibitor, emerging as a promising and safe therapy for chronic graft-versus-host disease patients. Methods This retrospective study describes the long-term outcomes of 23 chronic graft-versus-host disease patients treated with ruxolitinib. Results Most patients presented with severe chronic graft-versus-host disease (15/23; 65.2%). The overall response rate was 78.3% (18/23) after a median treatment duration of four weeks, with 55.6% (10/18) achieving complete response. At follow-up, 13 of the 18 responders (72.2%) sustained complete remission. Patients had a median of two previous lines of therapy, with a median follow-up of 14 months (range: 2-46 months) after starting ruxolitinib. Of the patients who were responsive to ruxolitinib, median follow-up extended to 26.5 months. Notably, for the patients who were responsive to ruxolitinib, the 1-year, 2-year, and 3-year overall survival was 83.3% (95% CI: 64.2%-102%), 56.1% (95% CI: 30.1%-80.9%), and 33.3% (95% CI: 9.2%-57.4%), respectively. Malignancy relapse occurred in 17.4% (4/23) of patients, with 34.7% (8/23) experiencing cytopenias, albeit mostly mild. Reactivation rates for cytomegalovirus were nil. Conclusion The long-term follow-up in this study supports ruxolitinib as an effective salvage therapy for chronic graft-versus-host disease with a 78.3% overall response rate and 55.6% complete remission rate. However, large prospective studies are warranted to validate these findings
Original article
The impact of pathogen reduction on ABO isoagglutinin titers in apheresis platelets Yeghiazaryan, Mikayel Ahmad, Yembur Singer, Jessie Nazaryan, Vaanush Fletcher, Craig Akgun, Yamac

Resumo em Inglês:

Abstract Background Platelet transfusions are a cornerstone of modern medical care, used across various clinical contexts. Ensuring the compatibility of blood products, especially regarding ABO isoagglutinins, is critical to minimize adverse reactions. Pathogen reduction technologies have been widely adopted to enhance the safety of blood products, however, the impact of such treatments on ABO isoagglutinin titers in platelet products remains unclear. Methods This study analyzed 60 apheresis platelet donations, including type O, A, and B donors, using the INTERCEPT® Blood System for pathogen reduction. Samples were collected both from donor whole blood at the time of apheresis (Retention) and from the final pathogen-reduced platelet product after it had passed through the compound adsorption device (Post-CAD). ABO isoagglutinin titers, including both IgM and IgG classes, were measured using solid-phase technology on the NEO Iris platform. Results This study found a significant reduction in IgM isoagglutinin titers in Post-CAD samples, with 99 % of Retention titers being greater than or equal to their Post-CAD counterparts. IgG titers exhibited more variability, with 9 % of Post-CAD samples displaying higher titers than Retention samples. Statistical analysis confirmed differences between Retention and Post-CAD samples for both IgM and IgG titers, with p-values <0.05 in most comparisons. Conclusion Pathogen reduction using the INTERCEPT® Blood System effectively reduces ABO isoagglutinin titers in apheresis platelets, potentially lowering the risk of hemolytic transfusion reactions. This reduction is beneficial for safer out-of-group platelet transfusions, especially in vulnerable populations such as pediatric patients. These findings support the continued use of pathogen-reduced platelets in transfusion medicine to enhance both safety and availability of blood products.
Original article
Clinical characteristics and outcomes of non-tuberculous mycobacterial pulmonary infections after hematopoietic stem cell transplantation: A retrospective cohort study Esber, Zahia Salam, Hamza Godara, Shefali Soubani, Ayman

Resumo em Inglês:

Abstract Introduction Non-tuberculous mycobacterial infections are rising as complications of bone marrow transplantation with lung disease being the most common clinical presentation. The identification and management of these infections in hematopoietic stem cell transplantation patients remains underrecognized. This study aims to investigate the clinical characteristics and outcomes in patients with post-transplant pulmonary infections. Methods The charts of 3,000 adult patients who received transplants over 11 years at the Karmanos Cancer Institute, a tertiary-care cancer center in Detroit, were reviewed. The diagnoses of post-transplant pulmonary non-tuberculous mycobacterial infections of 51 patients were defined as definite, probable or possible based on the American Thoracic Society (ATS) and Centers for Disease Control and Prevention guidelines. The identified organisms were further characterized as rapid- or slow-growing mycobacteria. Clinical characteristics, risk factors, microbiologic data, therapy and outcomes of the patients were collected and analyzed. Results About half (n = 26; 51%) of the patients were identified with definite pulmonary infection. There was a trend of cardiovascular and pulmonary comorbidities in these patients. The majority (n = 44; 86.3%) were on steroid and immunosuppressive therapy in the setting of graft-versus-host disease. The most common presenting symptoms were a combination of change in cough and worsening shortness of breath. The most common radiologic pattern was nodular infiltrates in 15 (29.4%) patients. Mycobacterium avium complex was identified in 38 (74.5%) patients. The majority of patients with these infections (76.5%) did not receive antimycobacterial therapy. Survival was reported in 42 (82.4%) patients. Conclusion Outcomes vary significantly among non-tuberculous mycobacterial pulmonary infections based on mycobacterial species, rate of colonization and degree of immunosuppression. The prognosis is overall good due to slow growing mycobacteria. Prospective multicenter studies are required to further guide the management of these patients.
Original article
Hematological ratios and cytokine profiles in heterozygous beta-thalassemia Ciceri, Ana Carolina Marques Oliveira, Laura Eduarda de Richter, Ana Luísa Carvalho, José Antonio Mainardi de Lucena, Maylla Rodrigues Gomes, Guilherme Wataru Figueiredo, Maria Stella Santos, Magnun Nueldo Nunes dos Blaia-D'Avila, Vera Lúcia Nascimento Cançado, Rodolfo Delfini Guerra-Shinohara, Elvira Maria Paniz, Clóvis

Resumo em Inglês:

Abstract Introduction β-Thalassemia is defined by a reduced or complete absence of β-globin chain synthesis in hemoglobin, leading to hemolytic anemia. Heterozygous β-thalassemia, also known as β-thalassemia trait (hBTh), the mildest form of this anemia, typically does not cause symptoms in carriers. However, it may lead to changes in the immune system, including an increase in total leukocyte, neutrophil, and lymphocyte counts. Objective This study aimed to evaluate various immune and inflammation markers, including neutrophil/lymphocyte, derived neutrophil/lymphocyte, lymphocyte/monocyte, platelet/lymphocyte, neutrophil/platelet ratios, systemic immune-inflammation index, systemic inflammation response index, neutrophil/natural killer cell ratio (NNKR), and inflammatory cytokines in β-thalassemia trait carriers. Method A retrospective observational study was conducted, including 50 β-thalassemia trait individuals and 100 healthy controls. Results Leukocyte, neutrophil and reticulocyte counts, and interleukin 6 levels were higher in carriers compared to controls. Notably, the β-thalassemia trait group had increased neutrophil/platelet, neutrophil/lymphocyte and derived neutrophil/lymphocyte ratios, and the systemic immune-inflammation and systemic inflammation response indexes were higher compared to the controls. Conclusions β-thalassemia trait shows a more pronounced inflammatory profile as indicated by hematological ratios. These ratios, therefore are potentially cost-effective and easily applicable markers for monitoring patients with the β-thalassemia trait.
Original article
Efficacy, safety and satisfaction of using emicizumab in hemophilia A patients without factor VIII inhibitors: A systematic review Araujo, Isabela de Oliveira Suassuna, Lucas Fernandes Santos, Isabela Lima dos Rodrigues, Daniela de Oliveira Werneck

Resumo em Inglês:

Abstract Background Hemophilia A is a genetic disorder characterized by deficiency or dysfunction of the factor VIII clotting protein, leading to serious bleeding disorders. Conventional treatment involves the exogenous administration of factor VIII. However, this therapy faces significant challenges, including the development of inhibitors and the need for frequent intravenous administration. Emicizumab, a recombinant bispecific monoclonal antibody that can be administered subcutaneously, offers a novel therapeutic alternative by mimicking the action of factor VIII. Methods This systematic review evaluates the efficacy, safety, and patient satisfaction with emicizumab in patients with hemophilia A without inhibitors. A comprehensive literature search was conducted using the MEDLINE, SciELO, and LILACS databases. The included studies were original articles on the use of emicizumab in hemophilia A patients without inhibitors and reviews, short communications, expert comments, and case reports were excluded. Data extraction and analysis were performed using predefined criteria. Results A total of 471 articles were identified, with 28 meeting the inclusion criteria. Studies demonstrated robust evidence of the efficacy of emicizumab in reducing bleeding episodes, with significant reductions in the Annualized Bleeding Rate and Annualized Joint Bleeding Rate. Safety profiles were favorable, with mainly minor adverse events reported. High patient satisfaction scores highlighted improvements in quality of life and treatment adherence. Conclusion Emicizumab represents a significant advancement in hemophilia A treatment, offering superior efficacy, safety, and patient satisfaction compared to traditional therapies. Future research should focus on long-term outcomes and specific subpopulations to further validate these findings.
Original article
Effect of testosterone on blood-clotting markers in transsexual men Reis, Estella Thaisa Sontag dos Dias, Carla Maria Franco Vieira, Carolina Sales Nadai, Mariane Nunes Okano, Sérgio Henrique Pires Franceschini, Silvio Antônio Lara, Lúcia Alves da Silva

Resumo em Inglês:

Abstract Background The use of testosterone in gender-affirming hormone therapy for trans men is associated with several adverse effects. However, research on the risk of venous thromboembolism in this treatment remains limited and inconclusive. This study aimed to assess the impact of intramuscular testosterone on specific direct and indirect blood-clotting markers in trans men. Method Treatment of trans men without previous use of testosterone was followed up in a prospective observational study in a trans people healthcare service. Gender-affirming hormone therapy was initiated with intramuscular testosterone cypionate (Depo-Testosterone). The blood-clotting markers prothrombin time, activated partial thromboplastin time, d-dimer, antithrombin, and factors VIII and VII were evaluated before and 12 weeks after starting the medication. Results Nineteen trans men with a mean age of 23.7 ± 3.7 years were enrolled. After 12 weeks of hormone therapy, significant increases in weight (p-value = 0.002) and body mass index (p-value = 0.007) were observed in patients. Furthermore, there were significant increases of 830 % in serum testosterone (p-value = 0.000), 7 % in hemoglobin (p-value = 0.000) and 10 % in hematocrit (p-value = 0.001). Conversely, a 10 % decrease in high density lipoprotein cholesterol levels (p-value = 0.000), and 15 % decrease in Factor VII (p-value = 0.000) were detected. Conclusion Intramuscular testosterone in trans men was associated with increases in hematocrit, hemoglobin, and the body mass index, and decreases in high density lipoprotein cholesterol and Factor VII. Nevertheless, these variables remained within normal reference values. Long-term follow-up studies evaluating gender-affirming hormone therapy with testosterone are needed to determine adequate risk management of venous and arterial thromboembolism in this population.
Original article
Prevalence of malaria parasites among blood donors in two hospitals in Enugu metropolis, Nigeria Aluh, Samuel ThankGod Ubachukwu, Patience Obiageli Onah, Kyrian Ikenna Oladepo, Gabriel Adebayo Ukwen, Chidi Ole Rimamkirnde, Fupsin

Resumo em Inglês:

Abstract Introduction Screening of blood donors for malaria parasites as recommended by the World Health Organization (WHO) is currently not included in the protocols and procedures for pre-screening blood donors of many private and public health facilities in Nigeria. Methods A cross-sectional study was conducted of voluntary, family, and remunerated blood donors in two hospitals in the Enugu metropolis. A well-structured questionnaire was used to collect demographics and blood donation history data. Five milliliters of blood were collected from each blood donor, of which 2 mL were used to screen for malaria parasites. Results Three hundred and seventy-seven blood donors participated in the study with 148 (39.3 %) being malaria-positive. Most of the blood donors were in the age groups 16-25 and 26-35 years old with prevalences of 40.0 % and 44.1 %, respectively. The prevalence of malaria in both age groups was high compared to the 36-45 years age group (26.7 %). Still, the overall difference in malaria prevalence across the four age groups was not statistically significant (χ2 = 5.437; p-value = 0.142). The majority (n = 290; 76.9 %) of the donors were male, while 87 (23.1 %) were female. Although female blood donors had a higher prevalence of malaria (47.1 %) compared to male donors (36.9 %), the difference was not statistically significant (p-value = 0.057). Conclusion The high prevalence of malaria in the studied area, suggests the need for careful screening of blood samples of blood donors for malaria parasites.
Original article
There is no transfer of mitochondria from donor hematopoietic cells to recipient mesenchymal stromal cells after allogeneic hematopoietic stem cells transplantation in humans Sats, Natalya Surin, Vadim Abramova, Tatiana Sadovskaya, Aleksandra Petinati, Nataliya Kapranov, Nikolay Nikiforova, Ksenia Drize, Nina Karaseva, Luisa Pokrovskaya, Olga Kuzmina, Larisa Parovichnikova, Elena

Resumo em Inglês:

Abstract Introduction Multipotent mesenchymal stromal cells are progenitors of the bone marrow stromal microenvironment that support hematopoiesis. Mitochondria, which can be transferred between cells via nanotubes or extracellular vesicles, play a key role in the functions of mesenchymal stromal cells. In a murine model, donor hematopoietic stem and progenitor cells transfer functional mitochondria to bone marrow mesenchymal stromal cells of the recipient. The aim of this study was to find out whether such transfer occurs in humans after allogeneic hematopoietic stem cell transplantation. Methods This study included nine patients with acute leukemia who received a reduced intensity conditioning regimen. Donor hematopoietic stem and progenitor cells mobilized into peripheral blood were the source of transplanted stem cells. Total DNA was isolated from bone marrow mesenchymal stromal cells of each patient before and after transplantation and their respective donors’ leukocytes. A fragment of mitochondrial DNA including the full-length control region was sequenced. The mitochondrial DNA sequence of each patient’s mesenchymal stromal cells was compared before and after the procedure and with the respective donor leukocytes. Results Donor mitochondrial DNA was not detected in the mesenchymal stromal cells of any patient after transplantation even as trace amounts. Co-culturing donor leukocytes with intact and irradiated mesenchymal stromal cells in vitro did not lead to detection of donor mitochondrial DNA transfer. Conclusion The data show that there is no mitochondrial transfer from donor hematopoietic stem and progenitor cells to recipient mesenchymal stromal cells after transplantation. Thus, the results indicate that one cannot count on improved mesenchymal stromal cell metabolism due to mitochondrial transfer. It is necessary to look for other ways to restore the stromal microenvironment.
Original article
Risk factors associated with HIV infection in four large Brazilian blood centers: A multicentric case-control study (2009-2017) Gonçalves, Fernanda Dominique de Souza Silva, Flávia da Costa Moura, Isabel Cristina Gomes Almeida-Neto, Cesar de Custer, Brian Sabino, Ester Cerdeira Loureiro, Paula Miranda, Carolina Amorim Filho, Luiz de Melo Salomon, Tassila

Resumo em Inglês:

Abstract Background Strategies to reduce contamination by transfusion-transmissible infections are constantly evolving. Over the years, HIV residual risk has decreased in several countries. However, in Brazil a recent study showed that the residual risk remains substantially higher than in other countries. Continuous surveillance of risk behaviors for infection in donors can help in pre-donation screening to reduce the risk of HIV in blood transfusions. Methods This analysis evaluated risk factors related to HIV infection among blood donors from four large Brazilian blood centers located in São Paulo, Rio de Janeiro, Belo Horizonte and Recife, from 2009-2017. A binary logistic model was used to evaluate any association between risk characteristics and behaviors and the occurrence of HIV. The significant variables were included in a saturated model, to which the backward strategy was applied to arrive at the final model. The analyses were carried out using the R program version 4.1.2 and p-value <0.05 was considered significant. Results A total of 1507 blood donors were included in the study, 716 were HIV positive and 791 were uninfected controls. Demographics significantly associated with infection were: Male sex, incomplete secondary education, separated/divorced/widowed, and bisexual/homosexual orientation. Behaviors most strongly associated with infection were: workplace exposure, intravenous drugs and men who had sex with other men. Conclusion The risk factors identified suggest that the blood donor screening process in Brazilian blood centers does not adequately identify donors at increased risk for HIV and further studies should be carried out to support changes to improve the process.
Original article
Translation, cross-cultural adaptation, and validation of the HCT frailty scale for hematopoietic stem cell transplant candidates: an observational study Lorca, Luz Larrain, Barbara Puga Ribeiro, Ivana Leao Valdivia, Ivana Gonzalez Hermoso, Angelia Fernández Maturana, Francisca Bass Lazcano, Francisco Canelo

Resumo em Inglês:

Abstract Introduction Hematopoietic stem cell transplantation (HSCT) is a treatment option for patients with hematologic malignancies. The aim of this study is to validate the Hematopoietic Cell Transplantation Frailty Scale in a Chilean population. Methods This was a cross-sectional scale validation study. The sample consisted of patients with various hematologic malignancies who were transplantation candidates. The study had two stages: (1) translation (forward and backward) and (2) psychometric analysis, including face validity, test-retest reliability, and content validity. Descriptive analyses included mean, standard deviation, and the 95 % confidence interval. Reliability was assessed with Spearman's correlation, and content validity used Kendall's W test. Results Fifty-four patients (53.7 % women) were included, with multiple myeloma being the most frequent diagnosis (33.3 %). Positive and strong correlations were identified (Spearman's Rho [ρ]: 1.0; p-value <0.001) for all items on the scale. Regarding content validity, there was agreement among evaluators for the categories of relevance and coherence (p-value <0.01; Kendall's W range: 0.13-0.17) but not for “clarity” (p-value = 0.11; Kendall's W: 0.07). Some terms in the content were adjusted without affecting the overall structure of the scale. In the retest analysis, descriptive values were similar to the initial test. Conclusion The Spanish version of the Hematopoietic Cell Transplantation Frailty Scale for Chile is conceptually and linguistically equivalent to the original instrument. Additionally, it demonstrated adequate psychometric properties in terms of validity and reliability.
Original article
Incidence of hepatocellular carcinoma in beta thalassemia: a systematic review and meta-analysis Adisuhanto, Marcella Prasetya, Alver Cahyadi, Alius Oehadian, Amaylia

Resumo em Inglês:

Abstract Background Current evidence indicates that iron overload increases the risk of hepatocellular carcinoma. However, the incidence of hepatocellular carcinoma in thalassemia is still unclear. This review aims to summarize the current evidence regarding the incidence of hepatocellular carcinoma in thalassemia patients. Methods Detailed searches were conducted in several databases, including PubMed, Europe PMC, EBSCOHost, and ProQuest. Keywords such as “thalassemia” and “hepatocellular carcinoma,” along with other relevant synonyms, were used. Articles investigating the incidence of hepatocellular carcinoma in thalassemia patients were included. Pooled estimates were calculated using the DerSimonian Laird inverse-variance random effect model and presented as incidence (%) along with their 95 % confidence intervals and 95 % prediction intervals. Results From a total of 318 articles, five studies encompassing a total of 9592 thalassemia patients were included in this study. The cumulative incidence of hepatocellular carcinoma in thalassemia patients was 1.96 % (95 % confidence interval: 0.88 %-4.27 %; prediction interval: 0.12 %-24.74 %; I2 = 86.8 %). Of the 139 hepatocellular carcinoma patients, 121 were reported positive for anti-HCV, 78 for HCV RNA, three for HbsAg, and 50 positive for anti-HBV or had past infections. The liver iron concentration and ferritin level ranges in all studies were 2.95-10.5 mg/g and 3.1-2950 µg/L, respectively. Conclusions The present meta-analysis demonstrates that the incidence of hepatocellular carcinoma in thalassemia patients was high (1.96 %). It might be caused by liver infection, iron overload, or something else.
Original article
Epidemiological characterization of chronic myeloid leukaemia patients at an oncologic centre: A retrospective observational study Meireles, Ana Maria Calisto, Rita Bento, Maria José Gouveia, Pedro Martinho Bizarro, Susana Teixeira, Manuel Moreira, Cláudia Santo, Ana Espírito Mariz, Mário

Resumo em Inglês:

Abstract Background The chronic myeloid leukaemia population, treatment patterns and responses in Portugal are unknown. The aim of this study is to describe these features in a Portuguese reference centre. Methods A retrospective cohort study included patients with chronic myeloid leukaemia, treated between 2012 and 2022 at the Instituto Português de Oncologia of Porto. Data were obtained from the Cancer Registry of the institution and clinical records. Variables included demographic data, treatments administered, responses (hematologic, cytogenetic, major and deep molecular responses), adverse events, and survival. Patients without available data, those treated in a clinical trial context, and those admitted only for hematopoietic transplantation were excluded. Results Ninety-nine patients were included in this study, with a median age of 52 years (range: 7-84 years) at diagnosis. The first-line treatment was imatinib in 96 patients however 33 required second-line with dasatinib, and 17 discontinued treatment while maintaining response. Regarding responses, 95 (96 %) patients achieved cytogenetic response, 90 (94 %) achieved major molecular response, and 71 (72 %) achieved deep molecular response. At three months, the early molecular response rate was 77 %. At 12 months of treatment, of the 67 patients with response evaluation, 93 % achieved complete cytogenetic response and 49 % major molecular response. Both imatinib and dasatinib were well tolerated. The median follow-up was eight years. The five-year overall survival was 96 %. Conclusion This study is the first to characterize chronic myeloid leukaemia patients at a Portuguese centre. The patient characteristics, responses, and overall survival were within the expected range according to the literature. This study confirms the good prognosis of chronic myeloid leukaemia and the good responses using imatinib as first-line treatment.
Original article
Oral post-surgical complications in patients with hemophilia and von Willebrand disease Sousa, Luisa Catarina Porfirio de Félix, Elanne Cristina Garcia da Costa Hespanhol, Wagner Pinheiro, Raquel dos Santos

Resumo em Inglês:

Abstract Objective To determine the prevalence of post-surgical complications in patients with hemophilia and von Willebrand disease. Methods A prospective, cross-sectional study with descriptive and exploratory data analysis was conducted at the outpatient clinic of the Arthur de Siqueira Cavalcanti State Institute of Hematology (Hemorio). The sample included 26 patients who underwent tooth extraction following the protocols of the Brazilian Ministry of Health. Results The prevalence of post-surgical complications identified in the study was 26.07 %, with 15.38 % of cases presenting bleeding after extraction. Conclusion The prevalence of postoperative complications found in this study was notably higher in patients with von Willebrand disease, followed by those with severe hemophilia.
Original article
Investigation of haematological, inflammatory parameters and the incidence of alloimmunization in multi-transfused sickle cell diseased patients Aboderin, Florence Ifechukwude Oduola, Taofeeq Davison, Glenda Mary Oguntibeju, Oluwafemi Omoniyi

Resumo em Inglês:

Abstract Introduction Sickle cell disease is a haemoglobinopathy caused by an aberrant mutation of the beta chain with the amino acid valine replacing glutamic acid at the 6th position. Patients with sickle cell disease suffer from complications including chronic inflammation and the development of allogeneic antibodies due to multiple blood transfusions. This study investigated the association between haematological, inflammatory markers and alloimmunization in multi-transfused patients with sickle cell disease. Methods This was a cross-sectional study, that enrolled 100 participants; 50 young adults (18-48 years) with homozygous sickle cell disease (Sickle cell Group) from the Obafemi University Health Centre in Nigeria, and 50 age and sex matched individuals who did not have the disease (Control group) but who had also received blood transfusions. Complete blood counts and differentials were processed on an auto-analyser (SFRI H18 Light, France). Red cell antigen identification used the saline and anti-human globin method while the abnormal haemoglobinopathy was evaluated using electrophoresis. ABO and Rhesus blood groups were analysed using a direct method on tile, and the determination of inflammatory markers including C-reactive protein, tumour necrosis factor-alpha, interleukin-6, and interleukin-1β was by the enzyme-linked immunosorbent assay technique. The data were statistically analysed using SPSS version 24.0 and GraphPad Prism. Additionally, the student t-test and Chi-square test were employed as appropriate. Data were presented as mean ± standard deviation, with a p-value <0.05 considered statistically significant. Result As expected, the Sickle Cell group had an increased rate of alloimmunisation and significantly reduced haemoglobin and red cell parameters except for the mean cell volume. Although both groups had platelet counts within the reference range the Sickle Cell group had significantly higher counts than the Control group. The Sickle Cell group displayed evidence of inflammation with significantly increased levels (p-value = 0.001) of C-reactive protein and tumour necrosis factor-alpha. This was supported by higher white cell counts and neutrophilia. The majority of the antibodies detected in sickle cell disease were anti-Kell, Jka and Fya while the controls showed a higher prevalence of anti-M and Kell antibodies. Despite the elevated inflammatory markers, no significant correlation was observed between these and the rate of alloimmunization. Conclusion In this study, the Sickle Cell group had an elevated rate of alloimmunization with higher levels of anti-kell, Jka and Fya as well as inflammatory markers. However, despite these findings, no significant correlation between inflammatory markers and alloimmunization could be detected. This suggests that elevated alloimmunization rates are multifactorial and involve other processes which require further investigation.
Review article
Blood storage effect of G6PD on RBC quality Cobbinah, Andrew Evans Sackey, Benedict Ofosu, Mina Dankluvi, Herbert Ekoe Opoku, Stephen Frank, Ampa Davis

Resumo em Inglês:

Abstract Background The most prevalent metabolic condition of red blood cells, glucose-6-phosphate dehydrogenase (G6PD) deficiency, affects around 35 million people globally. The highest prevalence is seen in tropical and subtropical areas of the eastern hemisphere, where it can affect up to 35 % of the population. G6PD deficiency, the most prevalent enzyme deficit, is not currently tested for in blood products. G6PD deficiency is a genetic factor that influences the quality of stored red blood cells impacting their ability to respond to oxidative stress. This hospital-based cross-sectional study aimed at assessing the prevalence of G6PD deficiency in donor blood and the impact of the enzyme deficiency on red cell indices during storage. Method A total of 57 blood bags were screened for G6PD deficiency. Red cell indices and blood film comments were investigated on Day 0, Day 7 and Day 14 of storage. Results Eight out of 57 (14 %) had the G6PD full defect and 86 % (49/57) had no defect. Over the course of 14 days storage, the hemoglobin and red blood cell count significantly decreased in G6PD-deficient blood units with a corresponding significant increase in mean corpuscular volume and red cell distribution width-standard deviation compared to baseline and normal G6PD activity. The blood film comment showed 85.7 % normocytic normochromic, 2.0 % microcytic hypochromic and 12.2 % macrocytic hyperchromic from G6PD-non-deficient donors whereas G6PD-deficient donors had 75 % normocytic normochromic with 12.5 % microcytic hypochromic and 12.5 % macrocytic hypochromic after 2 wk in storage. Conclusion Red blood cell count and hemoglobin reduce significantly in G6PD-deficient donor units during storage with an associated increased mean corpuscular volume indicating progressive loss of the cellular membrane homeostatic mechanism that could potentially result in further hemolysis during long term storage.
Review article
Advanced molecular approaches to thalassemia disorder and the selection of molecular-level diagnostic testing in resource-limited settings Meenakumari, Balaiah K, Chandramouleeswari Dhanasekar, Sariga

Resumo em Inglês:

Abstract Beta-thalassemia is a genetic disorder that significantly burdens healthcare systems globally. This inherited blood disorder, categorized into beta-thalassemia and alpha-thalassemia, results in insufficient globin production, leading to anemia and iron overload from frequent transfusions. Severe cases, known as thalassemia major, require regular blood transfusions. Beyond clinical suspicion and biochemical tests, molecular techniques are essential for confirming the diagnosis and guiding treatment. Advanced molecular profiling methods such as Polymerase Chain Reaction (PCR), Multiplex Ligation-dependent Probe Amplification (MLPA), Next-Generation Sequencing (NGS), Third-Generation Sequencing (TGS), and Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR) are effective in detecting mutations. Epigenetic factors also play a crucial role, driving the development of epidrugs for targeted therapy. This review covers various molecular techniques, established gene-editing methods, epigenetic mechanisms, and the impact of artificial intelligence on thalassemia management. It highlights the importance of selecting precise and sensitive molecular tools for detecting thalassemia gene mutations and stresses the need to make these testing methods accessible in resource-limited clinical settings.
Review article
CD36 as a marker of acute myeloid leukemia prognosis: A systematic review Amantéa, Marina Chaves Silva, Rafaela Pires da Soares, Larissa Ranini Pereira, João Lorenzo de Medeiros Souza, Ana Paula Duarte de

Resumo em Inglês:

Abstract CD36 is a glycoprotein associated with resistance to chemotherapy and the recurrence of acute myeloid leukemia. This systematic review aims to evaluate the impact of CD36 on the prognosis of acute myeloid leukemia, a complex heterogeneous malignant hematopoietic disease. The Embase, Scopus, Web of Science, Cochrane Library and SciELO databases were searched until September 2023. Only studies that analyzed CD36 expression in humans were included. Of 905 articles identified from the databases, 600 were screened and nine were included. The Newcastle-Ottawa Scale was used to evaluate the methodological quality of the studies. According to this systematic review, CD36 is associated with different prognostic factors in acute myeloid leukemia, including remission and relapse of the disease, overall survival, and chemoresistance.
Special article
A dream or reality: Consideration of ‘bloodless’ hematopoietic stem cell transplants for Jehovah’s witness patients MacNeill, Michael Fulford, Adrienne Caldwell, Deanna Deotare, Uday

Resumo em Inglês:

Abstract Background Hematopoietic stem cell transplantation (HSCT) is an important part of treatment for many hematologic conditions. The high-dose chemotherapy used in HSCTs puts patients at risk of significant cytopenias which often necessitate blood product transfusions. Certain populations, including Jehovah’s Witnesses, are unable to receive blood product transfusions during their transplant and thus, in the past, they have been seen as unsuitable candidates for transplantations. However, there has been growing evidence of the safety and efficacy of so-called “bloodless” HSCT protocols. Methods The most recent and relevant literature on “bloodless” transplants were identified through Embase, MEDLINE, and PubMed, and analyzed to construct a “bloodless” HSCT protocol at a Canadian centre. Since 2021, the regimen was utilized for four autologous transplantations in three different Jehovah’s Witness patients. Results None of the patients had a significant bleeding event nor a hemoglobin nadir below 8.0 g/dL. Minor bleeding events, predominantly mucositis, resolved with site-specific management. No patient had significant thrombocytopenia, and all the cell lines of patients had normalized without transfusions by the time of discharge. All patients were hospitalized for <30 days, similar to the experience of the centre with “regular” autologous transplants. Conclusion Careful planning and tailored regimens support the achievability of “bloodless” HSCTs in patients, such as Jehovah’s Witnesses, allowing practitioners to provide care to a previously excluded group and minimize the use of blood products in all HSCT patients.
Case Report
Influenza A-triggered Bickerstaff brainstem encephalitis successfully treated with therapeutic plasma exchange: A case report Czempik, Piotr F. Pięta, Małgorzata Jaworski, Tomasz Liberski, Piotr
Case Report
CAR T cell therapy-related lumbosacral polyradiculopathy with myelitis and stiff person syndrome with response to intravenous immunoglobulin and corticosteroids in a patient with acute lymphoblastic leukemia Goulart, Hannah Elmore, Kevin Scelsa, Stephen Park, Daniel Keyzner, Alla Ibrahim, Uroosa
Case Report
Onset of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis triggered by sudden reactivation of asymptomatic latent Epstein-Barr virus infection Mizuki, Masanari Terakawa, Takuya Umeki, Yuka Matsunaga, Hitomi Matsuoka, Yoshiki Nakahara, Wataru Matsui, Shogo Ikeda, Mako Ueshima, Kodai Hayasaka, Yuya Matsuoka, Nayu Tada, Yuma Matsui, Takahiro Oka, Kazumasa Ueda, Shuji
Letter to the Editor
Challenges in diagnosing thrombotic thrombocytopenic purpura Jacobs, Jeremy W. Booth, Garrett S. Adkins, Brian D.
Letter to the Editor
Cutaneous T-cell lymphomas may require an exception to the ABHH consensus regarding empiric vancomycin use in febrile neutropenia Gonzaga, Yung Sanches, Jose A.
Letter to the Editor
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Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular (ABHH) R. Dr. Diogo de Faria, 775 cj 133, 04037-002, São Paulo / SP - Brasil - São Paulo - SP - Brazil
E-mail: htct@abhh.org.br
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