Hematology, Transfusion and Cell Therapy最新文献

{"title":"Anti-Jra antibodies caused transiently positive free antibody test in a neonate in China: A case report and literature review","authors":"Yang Li , Yanjing He , Qiushi Wang , Wei Shen","doi":"10.1016/j.htct.2022.12.001","DOIUrl":"10.1016/j.htct.2022.12.001","url":null,"abstract":"","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S269-S273"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10364773","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pregnant women's knowledge and clinical management of hemolytic disease of the fetus and newborn in Pará, Brazil","authors":"Jhemily de Nazaré Gonçalves e Silva ,&nbsp;Andrya Maia de Souza ,&nbsp;Fabiane Monteiro do Rosario ,&nbsp;Laine Celestino Pinto","doi":"10.1016/j.htct.2024.03.007","DOIUrl":"10.1016/j.htct.2024.03.007","url":null,"abstract":"<div><h3>Objective</h3><div>To evaluate the knowledge of pregnant women and the clinical management of hemolytic disease of the fetus and newborn, as well as to describe the gestational profile, risk factors and socio-epidemiological profile of pregnant women treated at two municipal health units in Belém (Pará, Brazil).</div></div><div><h3>Methods</h3><div>This was a cross-sectional analytical study, which consisted in the application of questionnaires to pregnant women who underwent prenatal care at the municipal health units.</div></div><div><h3>Results</h3><div>A total of 104 pregnant women were evaluated; most were aged between 24 and 29 years old, had high school degrees (38 %), family incomes between 1 and 2 minimum wages (45 %) and blood type O+ (43 %). Regarding the gestational profile, the participants were predominantly in the third trimester of pregnancy (49 %), started prenatal care in the first gestational trimester (81 %) and were primiparous (61 %). Failures in the management of prenatal care were observed, especially with regard to access to information about the disease, since most pregnant women did not receive information about blood incompatibility during prenatal care. This led to limited knowledge about the pathology of the disease evidenced by the fact that most of the correct answers were between Questions 0–4, which were significantly associated with the women's education and income.</div></div><div><h3>Conclusions</h3><div>Although hemolytic disease of the fetus and newborn is serious, the pregnant women in this study demonstrated little knowledge about the disease and had inadequate care by health professionals, reinforcing the importance of improving care for women's health and prenatal care.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S201-S207"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141263578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of hepcidin-25/erythroferrone ratio as a potential biomarker for iron utility and erythropoiesis responsiveness to erythropoiesis-stimulating therapy in comparison to immature erythrocyte/reticulocyte parameters in hemodialysis patients","authors":"Salwa Bakr ,&nbsp;Karem Mohamed Salem ,&nbsp;Ahmed Mohammed Rashed ,&nbsp;Mohamed E A Tantawy ,&nbsp;Asmaa Younis Elsary ,&nbsp;Hanan Abdelmoneam Shamardl ,&nbsp;Eman Mahmoud Ezzat","doi":"10.1016/j.htct.2024.04.125","DOIUrl":"10.1016/j.htct.2024.04.125","url":null,"abstract":"<div><h3>Background</h3><div>Anemia-associated chronic kidney disease increases in more advanced stages with a subsequent acceleration in renal impairment progressing to end-stage renal disease. Although hepcidin and erythroferrone have been described as novel biomarkers of iron metabolism, there is still an area of ambiguity regarding iron utility in anemia-associated end-stage renal disease.</div></div><div><h3>Objectives</h3><div>This study aims to determine the correlations between erythropoietin, erythroferrone, and hepcidin-25 in hemodialysis, and to evaluate the clinical utility of the hepcidin-25/erythroferrone ratio as a biomarker of erythropoiesis-stimulating agent effectiveness compared to reticulocyte maturation parameters.</div></div><div><h3>Methods</h3><div>Serum erythropoietin, erythroferrone, and hepcidin-25 levels in 35 dialysis-dependent patients on a maintenance dose of a short-acting erythropoiesis-stimulating agent were consequently assessed on Days 0, 5, and 7. The erythropoiesis activity was monitored by measuring the increment in reticulocyte maturation parameters.</div></div><div><h3>Results</h3><div>Though the effectiveness of erythropoiesis in these patients was not associated with the hepcidin-25/erythroferrone ratio, it was lower among those with effective erythropoiesis than those with ineffective erythropoiesis. The effective group showed a statistically significant increase in reticulocyte maturation parameters compared to the ineffective group.</div></div><div><h3>Conclusions</h3><div>The findings show the pathogenesis of iron homeostasis in hemodialysis, the validity of hepcidin-25/erythroferrone ratio as a biomarker of erythropoiesis-stimulating agent effectiveness, and the advantageous monitoring of reticulocyte maturation measures to improve management of anemia-associated chronic kidney disease.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S214-S222"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142047700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes of thrombotic thrombocytopenic purpura patients submitted to therapeutic plasma exchange in a single center in Brazil","authors":"Patrícia Oliveira Cunha Terra ,&nbsp;Gil Cunha De Santis ,&nbsp;Benedito de Pina Almeida Prado Júnior ,&nbsp;Luciana Correa Oliveira","doi":"10.1016/j.htct.2023.11.006","DOIUrl":"10.1016/j.htct.2023.11.006","url":null,"abstract":"<div><h3>Introduction</h3><div>Immune thrombotic thrombocytopenic purpura (iTTP) is characterized by acute systemic microvascular thrombosis and is associated with a high morbidity and mortality, especially in delayed diagnosis (later than 6–7 days from symptoms). iTTP data in Brazil is scarce, so we aimed to characterize the clinical presentation and identify predictors of death risk in patients with this disease in Brazil.</div></div><div><h3>Methods</h3><div>In this single-center retrospective study the patients who underwent therapeutic plasma exchange (TPE) for presumptive or confirmed iTTP were evaluated regarding the epidemiological, clinical, laboratorial characteristics and management.</div></div><div><h3>Results</h3><div>A total of 50 patients (90 % female), with median age (IQR) of 34.1 (27–47) years, were enrolled, of which 12 (24 %) died. The most frequent symptoms were neurological (96 %), bleeding (76 %), gastrointestinal (52 %), fever (38 %), and cardiovascular (22 %). Neurological focal deficit and cardiovascular symptoms were more frequently observed in the non-survivor group (<em>P</em> = 0.0019 and <em>P</em> = 0.007, respectively). The mean ± SD number of days from beginning of symptoms to first TPE was 12.22 ± 7.91. We identified an association regarding mortality rate with a score MITS ≥ 2 points (<em>P</em> = 0.04), a higher indirect bilirubin (<em>P</em> = 0.0006), a higher number of transfused red blood cell units (<em>P</em> = 0.025), and platelet transfusion (<em>P</em> = 0.027).</div></div><div><h3>Conclusion</h3><div>Delayed diagnosis appears to be associated with a higher frequency of neurological symptoms and mortality. Intensity of hemolysis and signs of organ ischemia, such as cardiovascular symptoms and focal neurological deficit, are indicators of death risk.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S24-S31"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139189413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Research opportunities in Classical Hematology","authors":"Carla Roberta Peachazepi Moraes ,&nbsp;Edwin Mayr ,&nbsp;Erich Vinicius De Paula","doi":"10.1016/j.htct.2024.10.001","DOIUrl":"10.1016/j.htct.2024.10.001","url":null,"abstract":"","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S1-S2"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric autoimmune hemolytic anemia: A single-center retrospective study","authors":"Aline Sayuri Sakamoto,&nbsp;Fernanda Silva Sequeira,&nbsp;Bruna Paccola Blanco,&nbsp;Marlene Pereira Garanito","doi":"10.1016/j.htct.2023.12.006","DOIUrl":"10.1016/j.htct.2023.12.006","url":null,"abstract":"<div><h3>Background</h3><div>Autoimmune hemolytic anemia (AIHA) is a rare, life-threatening disease in pediatrics. This article describes the clinical features, diagnostic workup, treatment and outcome in patients with AIHA.</div></div><div><h3>Method</h3><div>Medical charts of under 18-year-old patients with AIHA treated at a tertiary Brazilian institution from 2006 to 2021 were retrospectively reviewed. Data analysis was primarily descriptive, using medians, interquartile ranges, and categorical variables presented as absolute frequencies.</div></div><div><h3>Main results</h3><div>Twenty-four patients (14 female, 10 male) were evaluated in this study. The median age at diagnosis was 5.99 years (range: 0.25–17.1 years) and the median hemoglobin level was 4.85 g/dL (range: 4.17–5.57 g/dL). Most had warm antibodies (83.3 %). Twelve patients (50 %) had known underlining diseases, four (16.6 %) presented with AIHA concomitant with acute infectious diseases and three (12.5 %) had an undetermined post-vaccine association. Steroids and intravenous immunoglobulin were first-line therapy in 23 cases. Seven patients (29.1 %) required second and third-line treatments (rituximab, cyclophosphamide and splenectomy). The median follow-up period was 4.4 years (range: 1.0–6.7 years). Thirteen patients (54.1 %) were discharged, five cases (20.8 %) were lost to follow-up and no patient died. The median age for the six remaining patients was 11.53 years (8.5–14.7) with all of them having complete responses with no further therapies.</div></div><div><h3>Conclusion</h3><div>Most cases of AIHA are secondary to an underlying systemic disease or have a possible correlation with infections/vaccines and respond to steroids. The second and third-line therapies for refractory and relapse cases remain a dilemma. A prospective, multicenter study is essential to address the best therapeutic combinations.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S181-S188"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139944811","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hemolytic disease of the fetus and newborn—a perspective of immunohematology","authors":"Mirelen Moura de Oliveira Rodrigues ,&nbsp;Denise Mattos ,&nbsp;Silvana Almeida ,&nbsp;Marilu Fiegenbaum","doi":"10.1016/j.htct.2024.04.122","DOIUrl":"10.1016/j.htct.2024.04.122","url":null,"abstract":"<div><h3>Background</h3><div>Hemolytic disease of the fetus and newborn is a public health problem caused by maternal-fetal incompatibility; no prophylaxis is available for most alloantibodies that induce this disease. This study reviews the literature regarding which antibodies are the most common in maternal plasma and which were involved in hemolytic disease of the fetus and newborn.</div></div><div><h3>Method</h3><div>Seventy-five studies were included in this review using a systematic search. Two independent authors identified studies of interest from the PubMed and SciELO databases.</div></div><div><h3>Main results</h3><div>Forty-four case reports were identified, of which 11 babies evolved to death. From 17 prevalence studies, the alloimmunization rate was 0.17 % with 161 babies receiving intrauterine transfusions and 23 receiving transfusions after birth. From 28 studies with alloimmunized pregnant women (7616 women), 455 babies received intrauterine transfusions and 21 received transfusions after birth.</div></div><div><h3>Conclusion</h3><div>Rh, Kell, and MNS were the commonest blood systems involved. The geographical distribution of studies shows that as these figures vary between continents, more studies should be performed in different countries. Investing in early diagnosis is important to manage the risks and complications of hemolytic disease of the fetus and newborn.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S246-S257"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142147188","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hemolytic anemia due to pyruvate kinase deficiency coexistent with the alpha thalassemia trait and chronic myeloid leukemia","authors":"Nirmal Shrestha,&nbsp;Harshit Khurana,&nbsp;Renjith Verghese,&nbsp;Yogendra Mishra","doi":"10.1016/j.htct.2024.04.130","DOIUrl":"10.1016/j.htct.2024.04.130","url":null,"abstract":"","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S305-S308"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142304630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epidemiology of immune thrombocytopenia: study of adult patients at a referral hematology service in Northeastern Brazil","authors":"Bruna Sobreira Kubrusly ,&nbsp;Elsie Sobreira Kubrusly ,&nbsp;Hermano Alexandre Lima Rocha ,&nbsp;Antonio Brazil Viana Júnior ,&nbsp;Marcela Sobreira Kubrusly ,&nbsp;Lucas Loiola Ponte Albuquerque Ribeiro ,&nbsp;Rosângela de Albuquerque Ribeiro ,&nbsp;Fernando Barroso Duarte","doi":"10.1016/j.htct.2023.09.2363","DOIUrl":"10.1016/j.htct.2023.09.2363","url":null,"abstract":"<div><div>Immune thrombocytopenia (ITP) is an acquired bleeding disorder observed in the clinical practice. Little is known about its epidemiology in Brazil. The present study was conducted at a hematology referral center which covers a population of over 8 million in 184 municipalities in the state of Ceará. The purpose of this study was to draw a demographic profile of adult ITP patients with regard to sex, age, geographical origin and distribution across the state, and the proportion of secondary ITP. Following ethics committee approval, information was collected with an <em>ad hoc</em> instrument. The sample consisted of 187 adult ITP patients attending the Walter Cantídio University Hospital in 2015. The median follow-up time was 67 months (range: 1 month to 29 years). Female sex (<em>n</em> = 154; 82.35 %) was strongly prevalent in all age brackets, with an overall female/male ratio of 4.7:1. The median age was 41 ± 16.1 with an interquartile range of 29–55.5 years; there was no difference between the genders. Secondary ITP (18/187; 9.6 %) displayed a bimodal distribution and a linear increase between 38 and &gt;68 years of age. The results of this survey on the epidemiology of ITP in Brazil suggest that ethnic and geographical factors may have a great impact on age and sex distribution and on the distribution of secondary ITP.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S152-S157"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139099424","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Minor surgical procedures during immune tolerance induction in people with hemophilia A and inhibitors: results from the Brazilian Immune Tolerance (BrazIT) study cohort","authors":"Ricardo Mesquita Camelo ,&nbsp;Maíse Moreira Dias ,&nbsp;Suely Meireles Rezende ,&nbsp;Brazilian Immune Tolerance (BrazIT) Study group","doi":"10.1016/j.htct.2024.05.010","DOIUrl":"10.1016/j.htct.2024.05.010","url":null,"abstract":"<div><h3>Introduction</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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.</div></div><div><h3>Conclusion</h3><div>No association was found between having a minor surgical procedure and ITI treatment outcome.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S223-S226"},"PeriodicalIF":1.8,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142304632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}