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Why do RhD negative pregnant women still become anti-D immunized despite prophylaxis with anti-D immunoglobulin? 为什么 RhD 阴性孕妇在使用抗 D 免疫球蛋白进行预防后,仍会出现抗 D 免疫反应?
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-07-02 DOI: 10.1016/j.transci.2024.103969
Çiğdem Akalın Akkök

Maternal allo-anti-D in RhD negative pregnant women may cause mild to severe hemolytic disease of the fetus and newborn. Although several other antibodies may also destroy red blood cells of the fetus and newborn, preventive measures with anti-D immunoglobulin are only available for D antigen. Targeted antenatal care together with postpartum prophylaxis with anti-D immunoglobulin has significantly reduced the D-alloimmunization risk. Potentially sensitizing events like trauma to the pregnant abdomen, vaginal bleeding, and amniocentesis may lead to fetomaternal hemorrhage and necessitate additional doses. Despite comprehensive programs with these targeted measures, allo-anti-D is still the most common reason for severe hemolytic disease of the fetus and newborn. Where do we fail then? Here, in this review, I would therefore like to discuss the reasons for D-alloimmunizations hoping that the greater focus will pave the way for further reduction in the number of pregnancy-related allo-anti-Ds.

RhD 阴性孕妇的母体同种抗 D 可能会导致胎儿和新生儿轻度到重度溶血病。虽然其他几种抗体也可能破坏胎儿和新生儿的红细胞,但只有 D 抗原才有抗 D 免疫球蛋白预防措施。有针对性的产前护理和产后使用抗 D 免疫球蛋白的预防措施大大降低了 D 抗原免疫的风险。潜在的致敏事件,如孕妇腹部创伤、阴道出血和羊膜穿刺术,可能会导致胎儿-产妇大出血,因此有必要追加剂量。尽管采取了这些有针对性的综合措施,但异抗原-D 仍是导致胎儿和新生儿严重溶血病的最常见原因。那么,我们失败在哪里呢?因此,在这篇综述中,我想讨论 D-同种抗原免疫的原因,希望更多的关注能为进一步减少与妊娠相关的同种抗原免疫铺平道路。
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引用次数: 0
Evaluation of transfusion reactions in patients following transfusion of blood components containing antibodies to HLA class I – An attempt to prevent TRALI in patients 评估输注含有 HLA I 类抗体的血液成分后患者的输血反应 - 预防患者 TRALI 的尝试。
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-07-02 DOI: 10.1016/j.transci.2024.103970
Nhan T. Nguyen, Thomas Larsen Titze, Lise Sofie H. Nissen-Meyer

In an attempt to mitigate transfusion-related acute lung injury (TRALI), the Oslo Blood Center screened 1369 thrombapheresis donors for human leucocyte antigen (HLA)-specific antibodies. Anti-HLA antibodies were found in 200 donors who were deferred from donation of plasma-rich products. In a retrospective study, 2562 transfusions of thrombocytes (both apheresis and whole blood-derived) from 150 of these donors were subject to a thorough look back-investigation. Reports of 14 transfusion reactions were identified, none of which were classified as TRALI. Our study supports previous data indicating that the risk of TRALI is low. The value of screening for anti-HLA antibodies and subsequent deferral of donors with high levels of such antibodies remains questionable.

为了减轻输血相关急性肺损伤(TRALI),奥斯陆血液中心对 1369 名血栓形成捐献者进行了人类白细胞抗原(HLA)特异性抗体筛查。在 200 名被推迟捐献富血浆产品的捐献者中发现了抗 HLA 抗体。在一项回顾性研究中,对其中 150 名捐献者的 2562 次血小板输注(包括无细胞血浆和全血)进行了彻底的回顾调查。共发现 14 例输血反应报告,其中没有一例被归类为 TRALI。我们的研究支持之前的数据,表明 TRALI 的风险很低。对抗 HLA 抗体进行筛查并随后推迟高水平抗 HLA 抗体捐献者的价值仍然值得怀疑。
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引用次数: 0
Recombinant human thrombopoietin improves platelet engraftment after autologous hematopoietic stem cell transplantation in patients with aggressive lymphoma 重组人血小板生成素可改善侵袭性淋巴瘤患者自体造血干细胞移植后的血小板移植情况
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-07-02 DOI: 10.1016/j.transci.2024.103972
Xi Qiu, Huawei Jiang, Xibin Xiao, Liansheng Huang, Yang Xu

Background

To explore the efficiency and safety of recombinant human thrombopoietin (TPO) on the platelet engraftment after autologous stem cell transplantation (ASCT) in patients with aggressive lymphoma.

Patients and methods

Forty patients were enrolled in a single-center, retrospective clinical trial from July 2019 with rhTPO administration when the platelet count ≤ 75 × 109/L after the reinfusion of stem cells. The hematopoietic reconstitution, platelet transfusion dependence, the cost and length of hospitalization, side effects and survival benefit were compared between the rhTPO group and the control group of 25 historic patients without rhTPO.

Results

The cumulative incidence of platelet engraftment in the rhTPO group was significantly higher since d+ 13 post-transplantation. But no difference of neutrophil engraftment was found. rhTPO was considered to influence the platelet engraftment independently by multivariate analysis. Subgroup analysis demonstrated that when the patients were older than 45 years old, male, at stage-IV as diagnosed and obtained PR after previous treatment, rhTPO was more recommended to facilitate platelet early engraftment after ASCT. Although rhTPO didn’t relieve the dependency of platelet transfusion, patients had the shorter length of hospitalization. And better OS was shown in the rhTPO group.

Conclusion

rhTPO improved platelet engraftment after ASCT with aggressive lymphoma, especially the ones older than 45 years old, male, at stage-IV as diagnosed and obtained PR after previous treatment. Although rhTPO didn’t lessen platelet transfusion dependence, the length and medical cost of hospitalization were reduced when rhTPO was involved. rhTPO was efficacy and safety which could be recommended after ASCT.

背景探讨重组人血小板生成素(TPO)对侵袭性淋巴瘤患者自体干细胞移植(ASCT)后血小板移植的有效性和安全性。患者和方法自2019年7月起,一项单中心、回顾性临床试验招募了40例患者,当干细胞再输注后血小板计数≤75×109/L时给予rhTPO。比较了rhTPO组与未使用rhTPO的25例历史性患者对照组的造血重建、血小板输血依赖性、住院费用和住院时间、副作用和生存获益。多变量分析认为 rhTPO 对血小板移植有独立影响。亚组分析表明,当患者年龄大于 45 岁、男性、诊断为 IV 期且既往治疗后获得 PR 时,更推荐使用 rhTPO 来促进 ASCT 后血小板的早期移植。虽然 rhTPO 并不能减轻患者对血小板输注的依赖,但患者的住院时间更短。结论rhTPO可改善侵袭性淋巴瘤患者ASCT后的血小板移植,尤其是年龄大于45岁、男性、诊断为IV期且既往治疗后获得PR的患者。虽然rhTPO并没有减少血小板输注的依赖性,但参与rhTPO治疗的患者住院时间和医疗费用都有所减少。
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引用次数: 0
Editorial comments: Two clinical viewpoints from Oslo 编辑评论:来自奥斯陆的两种临床观点。
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-07-02 DOI: 10.1016/j.transci.2024.103968
Jerard Seghatchian
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引用次数: 0
The effect of agitating buffy coats on platelet quality before soft spin 软旋前搅拌缓冲液对血小板质量的影响
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-06-30 DOI: 10.1016/j.transci.2024.103964
Shichun Wang , Qi Liu , Ronghua Diao , Zerong Wang , Yahan Fan , Shuguang Liu , Jie Liu , Shuming Zhao , Chunyan Yao

Background

Platelet plays a vital role in both physiological and pathological processes. However, the limited storage time of platelet in vitro poses an immense challenge for its applications because of the increased risk of bacterial contamination and platelet storage lesions. Agitation can inhibit lesions by facilitating continuous oxygenation of platelets and permitting excess carbon dioxide to be removed during storage. However, it is still not known whether agitating BCs gives a positive effect on platelet quality. Objectives: To evaluate the quality difference between platelet concentrates (PCs) from buffy coats (BCs) held rest and agitation.

Methods

Samples were withdrawn for cell count, blood gas analysis, free hemoglobin level, hypotonic shock response, maximum aggregation rate, activation marker expression (CD62P and CD42b) and coagulation function. Results: We found the PCs prepared from the agitating BCs had fewer residual WBCs, exhibited a better gas exchange ability, slower metabolism (higher pH, higher content glucose, and lower lactic acid levels), better hypotonic shock response, and lower levels of CD62P. The TEG-PC assays showed no difference in coagulation function. Conclusion: Our findings showed that BC can be agitated overnight before a soft spin.

背景:血小板在生理和病理过程中都发挥着重要作用。然而,血小板在体外的储存时间有限,这给其应用带来了巨大挑战,因为细菌污染和血小板储存病变的风险会增加。搅拌可抑制病变,因为搅拌可促进血小板持续充氧,并允许在储存期间排出多余的二氧化碳。然而,搅拌 BC 是否会对血小板质量产生积极影响仍是未知数:目的:评估静置和搅拌状态下来自缓冲液(BCs)的血小板浓缩物(PCs)的质量差异:抽取样本进行细胞计数、血气分析、游离血红蛋白水平、低渗休克反应、最大聚集率、活化标志物表达(CD62P 和 CD42b)和凝血功能检测:结果:我们发现用搅拌 BC 制备的 PC 具有较少的残留白细胞、较好的气体交换能力、较慢的新陈代谢(较高的 pH 值、较高的葡萄糖含量和较低的乳酸水平)、较好的低张性休克反应和较低的 CD62P 水平。TEG-PC检测结果显示凝血功能没有差异:我们的研究结果表明,BC 可以在软旋转前搅拌过夜。
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引用次数: 0
Bacterial species in cord blood and their significance in the context of clinical use 脐带血中的细菌种类及其临床应用意义。
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-06-29 DOI: 10.1016/j.transci.2024.103961
Helena Barreto Henriksson , Ann Hellström , Anders K. Nilsson , Ulrika Sjöbom , Bodil Jönsson , Sofia Frändberg

Approximately 90 % of infants born before 28 full weeks(extremely-preterm-infants) receive erythrocyte transfusions in early life. Umbilical cord blood(UCB) has been investigated as an alternative source for erythrocyte transfusions to preterm neonates. This retrospective study aimed to compile/evaluate spectrum of bacteria groups/species intermittently detected in processed UCB at National-Swedish-Cord blood bank, (NS-CBB) during the years 2008–2020.

Consecutive data from the years 2008–2020 were investigated. UCB from healthy newborns born after 37 full weeks of gestation was collected following clamping of cord (1 min) through cannulation of umbilical vein(vaginal-and C-section-deliveries). In total, 5194 cord blood units (UCBUs) that met NS-CBB-guidelines for total nucleated-cell-content(TNC) were manufactured from 8875 collections. Of 5194 UCBUs,77,6 % were from vaginal-and 22,4 % from C-section deliveries.Samples(10 mL) were collected from surplus eryhtrocyte fraction post-processing(n = 5194), transferred into BACT/ALERT® aerobic/anaerobic culture flasks and monitored 10 days using BACT/ALERT®−3D-Microbial-Detection-Systems. Positive samples were subcultured and typed for bacterial groups and/or species.

Out of 5194 processed sampled UCB units,186 (3,6 %) were discarded due to positive sterility tests, 92 % were detected in samples from vaginal-deliveries and 8 % from C-section-deliveries. In all,16 different groups of bacteria and 27 species were identified. Common bacterial/groups and species were anaerobe gram-negative rods(n = 28),coagulase-negative-staphylococci(n = 21),gram-positive rods(n = 21),anaerobe-gram-positive cocci(n = 20) and viridans-streptococci(n = 13). Extracted from these results,in positive samples(n = 13) from C-section deliveries, bacteria were found:viridans-streptococci(n = 7),Aerococcus-urinae(n = 1), Staphylococcus lugdunensis(n = 1),other coagulase-negative staphylococci(n = 1) or a mix of aerobic/anaerobic bacteria(n = 3).

Our results are in alignment with previously published contamination rates in processed UCBUs. Still, results point towards importance of strict microbial monitoring when manufacturing UCBUs to achieve patient-safe- products for stem-cell transplantation/transfusion.

大约 90% 在出生满 28 周前出生的婴儿(极早产儿)在生命早期接受过红细胞输注。脐带血(UCB)作为早产新生儿输注红细胞的替代来源已得到研究。这项回顾性研究旨在整理/评估 2008-2020 年间在瑞典国家脐带血库(NS-CBB)处理过的脐带血中间歇性检测到的细菌群/物种谱。对 2008-2020 年间的连续数据进行了调查。通过脐静脉插管夹闭脐带(1 分钟)后,收集妊娠满 37 周后出生的健康新生儿(阴道和剖腹产)的脐带血。从 8875 份采集的脐带血中,共制成了 5194 份符合 NS-CBB 总核细胞含量(TNC)标准的脐带血单位(UCBU)。在 5194 个 UCBU 中,77.6% 来自阴道分娩,22.4% 来自剖腹产。从处理后的剩余红细胞部分(n = 5194)采集样本(10 mL),转移到 BACT/ALERT® 需氧/厌氧培养瓶中,并使用 BACT/ALERT®-3D 微生物检测系统进行为期 10 天的监测。对阳性样本进行亚培养,并进行细菌群和/或物种分型。在处理的 5194 个 UCB 单位样本中,有 186 个(3.6%)因无菌检测呈阳性而被丢弃,其中 92% 的样本来自阴道分娩,8% 来自剖腹产。总共确定了 16 个不同的细菌群和 27 个物种。常见的细菌/菌群和菌种为厌氧革兰阴性杆菌(28 个)、凝固酶阴性葡萄球菌(21 个)、革兰阳性杆菌(21 个)、厌氧革兰阳性球菌(20 个)和病毒链球菌(13 个)。根据这些结果,在剖腹产的阳性样本(n = 13)中发现的细菌有:病毒链球菌(n = 7)、尿气球菌(n = 1)、卢格登金葡萄球菌(n = 1)、其他凝固酶阴性葡萄球菌(n = 1)或需氧/厌氧菌混合菌(n = 3)。我们的结果与之前公布的加工过的 UCBU 污染率一致。尽管如此,结果表明,在生产UCBU时,必须严格监控微生物,以便为干细胞移植/输血提供对患者安全的产品。
{"title":"Bacterial species in cord blood and their significance in the context of clinical use","authors":"Helena Barreto Henriksson ,&nbsp;Ann Hellström ,&nbsp;Anders K. Nilsson ,&nbsp;Ulrika Sjöbom ,&nbsp;Bodil Jönsson ,&nbsp;Sofia Frändberg","doi":"10.1016/j.transci.2024.103961","DOIUrl":"10.1016/j.transci.2024.103961","url":null,"abstract":"<div><p>Approximately 90 % of infants born before 28 full weeks(extremely-preterm-infants) receive erythrocyte transfusions in early life. Umbilical cord blood(UCB) has been investigated as an alternative source for erythrocyte transfusions to preterm neonates. This retrospective study aimed to compile/evaluate spectrum of bacteria groups/species intermittently detected in processed UCB at National-Swedish-Cord blood bank, (NS-CBB) during the years 2008–2020.</p><p>Consecutive data from the years 2008–2020 were investigated. UCB from healthy newborns born after 37 full weeks of gestation was collected following clamping of cord (1 min) through cannulation of umbilical vein(vaginal-and C-section-deliveries). In total, 5194 cord blood units (UCBUs) that met NS-CBB-guidelines for total nucleated-cell-content(TNC) were manufactured from 8875 collections. Of 5194 UCBUs,77,6 % were from vaginal-and 22,4 % from C-section deliveries.Samples(10 mL) were collected from surplus eryhtrocyte fraction post-processing(n = 5194), transferred into BACT/ALERT® aerobic/anaerobic culture flasks and monitored 10 days using BACT/ALERT®−3D-Microbial-Detection-Systems. Positive samples were subcultured and typed for bacterial groups and/or species.</p><p>Out of 5194 processed sampled UCB units,186 (3,6 %) were discarded due to positive sterility tests, 92 % were detected in samples from vaginal-deliveries and 8 % from C-section-deliveries. In all,16 different groups of bacteria and 27 species were identified. Common bacterial/groups and species were anaerobe gram-negative rods(n = 28),coagulase-negative-staphylococci(n = 21),gram-positive rods(n = 21),anaerobe-gram-positive cocci(n = 20) and viridans-streptococci(n = 13). Extracted from these results,in positive samples(n = 13) from C-section deliveries, bacteria were found:<em>viridans-streptococci</em>(n = 7),<em>Aerococcus-urinae</em>(n = 1), <em>Staphylococcus lugdunensis</em>(n = 1)<em>,</em>other coagulase-negative staphylococci(n = 1) or a mix of aerobic/anaerobic bacteria(n = 3).</p><p>Our results are in alignment with previously published contamination rates in processed UCBUs. Still, results point towards importance of strict microbial monitoring when manufacturing UCBUs to achieve patient-safe- products for stem-cell transplantation/transfusion.</p></div>","PeriodicalId":49422,"journal":{"name":"Transfusion and Apheresis Science","volume":"63 4","pages":"Article 103961"},"PeriodicalIF":1.4,"publicationDate":"2024-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1473050224001290/pdfft?md5=adb786004bdd4be33a5f9067ba448522&pid=1-s2.0-S1473050224001290-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141564946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Irregular red blood cell antibodies, abnormal hemoglobin and dangerous universal blood donor insights from a public blood center in a Brazilian metropolitan area 巴西大都会地区一家公共血液中心的不规则红细胞抗体、异常血红蛋白和危险的通用献血者见解。
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-06-21 DOI: 10.1016/j.transci.2024.103963
Laiane da Silva Santos , Sérgio Eduardo Soares Fernandes , Anna Luiza Oliveira Sant’Anna , Flávio Ferreira Pontes Amorim , Felipe Ferreira Pontes Amorim , Fábio Ferreira Amorim

Background

Immunohematology tests are crucial in transfusion safety. This study aimed to assess irregular red blood cell (RBC) antibodies, abnormal hemoglobin and dangerous universal blood donors at a public blood center in a Brazilian metropolitan area.

Methods

A cross-sectional study included all consecutive blood donors from January 2018 to December 2021 at the Brasília Blood Center Foundation, Federal District (FD), Brazil.

Results

Among 205,965 blood donations, irregular RBC antibodies were found in 743 (0.4 %). Abnormal hemoglobin was observed in 5396 (2.6 %): 3959 (1.9 %) with Hb AS, 1344 (0.7 %) with Hb AC, and 93 (< 0,1 %) with other hemoglobin variants. Of O group donors, 12.5 % (9646) had hemolysins: 12.5 % (2410) both anti-A and anti-B, 8.7 % (9646) only anti-A, and 1.6 % (1763) only anti-B hemolysins. Female sex (p < 0.001) and increasing age (p < 0.001) were associated with irregular RBC antibodies. O and/or Rh(D)-positive blood groups had a lower prevalence of irregular RBC antibodies compared to other ABO and/or Rh(D)-negative groups. Age (p < 0.001) and female sex (p < 0.001) were associated with anti-A/anti-B hemolysins, while FD residency was associated with reduced incidence (p < 0.001).

Conclusion

Anti-A/anti-B hemolysins in O group donors, abnormal hemoglobin and irregular RBC antibodies pose risks to transfusion practice and should not be overlooked. Advancing age, female sex, ABO blood group other than O, or Rh(D)- negative are independently associated with the presence of irregular RBC antibodies. Dangerous universal blood donors were associated with advanced age, female gender, Rh(D)-positive blood type, and individuals residing in a Brazilian state other than where the blood center was located.

背景:免疫血液学检测对输血安全至关重要。本研究旨在评估巴西大都会地区一家公共血液中心的不规则红细胞(RBC)抗体、异常血红蛋白和危险的全民献血者:一项横断面研究纳入了巴西联邦区巴西利亚血液中心基金会从2018年1月至2021年12月的所有连续献血者:在205965名献血者中,有743人(0.4%)发现了不规则的红细胞抗体。5396人(2.6%)血红蛋白异常:3959人(1.9%)的血红蛋白为AS型,1344人(0.7%)的血红蛋白为AC型,93人(结论:血红蛋白为AS型和AC型)的血红蛋白为B型:O 组献血者中的抗-A/抗-B 溶血素、异常血红蛋白和不规则红细胞抗体给输血实践带来风险,不容忽视。年龄增长、女性性别、ABO 血型非 O 型或 Rh(D)- 阴性与不规则红细胞抗体的存在有独立关联。危险的全民献血者与高龄、女性、Rh(D)阳性血型以及居住在血液中心所在地以外的巴西其他州有关。
{"title":"Irregular red blood cell antibodies, abnormal hemoglobin and dangerous universal blood donor insights from a public blood center in a Brazilian metropolitan area","authors":"Laiane da Silva Santos ,&nbsp;Sérgio Eduardo Soares Fernandes ,&nbsp;Anna Luiza Oliveira Sant’Anna ,&nbsp;Flávio Ferreira Pontes Amorim ,&nbsp;Felipe Ferreira Pontes Amorim ,&nbsp;Fábio Ferreira Amorim","doi":"10.1016/j.transci.2024.103963","DOIUrl":"10.1016/j.transci.2024.103963","url":null,"abstract":"<div><h3>Background</h3><p>Immunohematology tests are crucial in transfusion safety. This study aimed to assess irregular red blood cell (RBC) antibodies, abnormal hemoglobin and dangerous universal blood donors at a public blood center in a Brazilian metropolitan area.</p></div><div><h3>Methods</h3><p>A cross-sectional study included all consecutive blood donors from January 2018 to December 2021 at the Brasília Blood Center Foundation, Federal District (FD), Brazil.</p></div><div><h3>Results</h3><p>Among 205,965 blood donations, irregular RBC antibodies were found in 743 (0.4 %). Abnormal hemoglobin was observed in 5396 (2.6 %): 3959 (1.9 %) with Hb AS, 1344 (0.7 %) with Hb AC, and 93 (&lt; 0,1 %) with other hemoglobin variants. Of O group donors, 12.5 % (9646) had hemolysins: 12.5 % (2410) both anti-A and anti-B, 8.7 % (9646) only anti-A, and 1.6 % (1763) only anti-B hemolysins. Female sex (p &lt; 0.001) and increasing age (p &lt; 0.001) were associated with irregular RBC antibodies. O and/or Rh(D)-positive blood groups had a lower prevalence of irregular RBC antibodies compared to other ABO and/or Rh(D)-negative groups. Age (p &lt; 0.001) and female sex (p &lt; 0.001) were associated with anti-A/anti-B hemolysins, while FD residency was associated with reduced incidence (p &lt; 0.001).</p></div><div><h3>Conclusion</h3><p>Anti-A/anti-B hemolysins in O group donors, abnormal hemoglobin and irregular RBC antibodies pose risks to transfusion practice and should not be overlooked. Advancing age, female sex, ABO blood group other than O, or Rh(D)- negative are independently associated with the presence of irregular RBC antibodies. Dangerous universal blood donors were associated with advanced age, female gender, Rh(D)-positive blood type, and individuals residing in a Brazilian state other than where the blood center was located.</p></div>","PeriodicalId":49422,"journal":{"name":"Transfusion and Apheresis Science","volume":"63 4","pages":"Article 103963"},"PeriodicalIF":1.4,"publicationDate":"2024-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141538840","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Genotype-phenotype analyses of Iranian patients with hemophilia B (Leyden -) and hemophilia B (Leyden +): A single-center study 伊朗 B 型血友病(莱登-)和 B 型血友病(莱登+)患者的基因型-表型分析:单中心研究。
IF 1.4 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-06-20 DOI: 10.1016/j.transci.2024.103962
Arash Ahmadfard Moghadam , Amir Reza Manafzadeh , Khadijeh Dajliry , Farahnaz Ramezan , Mohammad Reza Nikoonia , Babak Abdolkarimi , Mohsen Hamidpour , Shadi Tabibian

Background

There is a high prevalence of inherited bleeding disorders in Iran, such as hemophilia A (HA) and hemophilia B (HB). This study aimed to analyze the molecular and clinical profiles of patients with HB.

Methods

A single-center study was conducted among patients with severe HB between March 20, 2000, and June 31, 2023. The polymerase chain reaction (PCR) amplification was used for all of the major regions, such as the promoter, the exons, the adjacent intronic regions, and the untranslated regions of the F9 gene. Finally, Sanger sequencing was performed on the PCR products.

Results

A total of 111 HB patients (17 with HB [Leyden +] and 94 with HB [Leyden -]) were enrolled in this study. Among 94 patients with HB (Leyden -), 59 (62.8 %) had missense, 21 (22.3 %) had nonsense, and 8 (8.5 %) had frameshift mutations. Moreover, the most frequent pathogenic variant in HB (Leyden +) was c.–17 A>G in this study.

Conclusion

The results of this study confirm that HB is caused by a wide range of molecular defects in Iran. Thus, by knowing the genotypes and phenotypes, we would be able to stratify the patients which is important in terms of their management and outcome.

背景:在伊朗,血友病 A(HA)和血友病 B(HB)等遗传性出血性疾病的发病率很高。本研究旨在分析 HB 患者的分子和临床特征:方法:对 2000 年 3 月 20 日至 2023 年 6 月 31 日期间的重症 HB 患者进行了单中心研究。采用聚合酶链反应(PCR)扩增 F9 基因的所有主要区域,如启动子、外显子、邻近的内含子区和非翻译区。最后,对 PCR 产物进行 Sanger 测序:本研究共纳入 111 例 HB 患者(17 例 HB [莱登 +]和 94 例 HB [莱登 -])。在94名HB(莱登-)患者中,59人(62.8%)存在错义突变,21人(22.3%)存在无义突变,8人(8.5%)存在移帧突变。此外,在本研究中,HB(莱登+)最常见的致病变异是c.-17 A>G:本研究结果证实,在伊朗,HB 是由多种分子缺陷引起的。因此,通过了解基因型和表型,我们可以对患者进行分层,这对患者的治疗和预后非常重要。
{"title":"Genotype-phenotype analyses of Iranian patients with hemophilia B (Leyden -) and hemophilia B (Leyden +): A single-center study","authors":"Arash Ahmadfard Moghadam ,&nbsp;Amir Reza Manafzadeh ,&nbsp;Khadijeh Dajliry ,&nbsp;Farahnaz Ramezan ,&nbsp;Mohammad Reza Nikoonia ,&nbsp;Babak Abdolkarimi ,&nbsp;Mohsen Hamidpour ,&nbsp;Shadi Tabibian","doi":"10.1016/j.transci.2024.103962","DOIUrl":"10.1016/j.transci.2024.103962","url":null,"abstract":"<div><h3>Background</h3><p>There is a high prevalence of inherited bleeding disorders in Iran, such as hemophilia A (HA) and hemophilia B (HB). This study aimed to analyze the molecular and clinical profiles of patients with HB.</p></div><div><h3>Methods</h3><p>A single-center study was conducted among patients with severe HB between March 20, 2000, and June 31, 2023. The polymerase chain reaction (PCR) amplification was used for all of the major regions, such as the promoter, the exons, the adjacent intronic regions, and the untranslated regions of the <em>F9</em> gene. Finally, Sanger sequencing was performed on the PCR products.</p></div><div><h3>Results</h3><p>A total of 111 HB patients (17 with HB [Leyden +] and 94 with HB [Leyden -]) were enrolled in this study. Among 94 patients with HB (Leyden -), 59 (62.8 %) had missense, 21 (22.3 %) had nonsense, and 8 (8.5 %) had frameshift mutations. Moreover, the most frequent pathogenic variant in HB (Leyden +) was c.–17 A&gt;G in this study.</p></div><div><h3>Conclusion</h3><p>The results of this study confirm that HB is caused by a wide range of molecular defects in Iran. Thus, by knowing the genotypes and phenotypes, we would be able to stratify the patients which is important in terms of their management and outcome.</p></div>","PeriodicalId":49422,"journal":{"name":"Transfusion and Apheresis Science","volume":"63 4","pages":"Article 103962"},"PeriodicalIF":1.4,"publicationDate":"2024-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The use of therapeutic plasma exchange in systemic loxoscelism induced treatment resistant hemolytic anemia: A case report 使用治疗性血浆置换治疗系统性红细胞增多症引起的耐药性溶血性贫血:病例报告
IF 1.9 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-06-14 DOI: 10.1016/j.transci.2024.103960
Joshua Mehr, Jaehyup Kim

Brown recluse spider bites can lead to severe reactions such as skin necrosis,hemolytic anemia, and multiorgan failure, which can be life-threatening. Therapeutic plasma exchange has been reported to provide clinical benefit for such cases. In thisreport, we present a case of a brown recluse spider bite that was successfully treated with therapeutic plasma exchange and compare it with previous case reports.

棕色隐士蛛咬伤可导致严重反应,如皮肤坏死、溶血性贫血和多器官衰竭,可危及生命。据报道,治疗性血浆置换可为此类病例带来临床益处。在本报告中,我们介绍了一例成功接受治疗性血浆置换治疗的棕色隐士蜘蛛咬伤病例,并将其与之前的病例报告进行了比较。
{"title":"The use of therapeutic plasma exchange in systemic loxoscelism induced treatment resistant hemolytic anemia: A case report","authors":"Joshua Mehr,&nbsp;Jaehyup Kim","doi":"10.1016/j.transci.2024.103960","DOIUrl":"https://doi.org/10.1016/j.transci.2024.103960","url":null,"abstract":"<div><p>Brown recluse spider bites can lead to severe reactions such as skin necrosis,hemolytic anemia, and multiorgan failure, which can be life-threatening. Therapeutic plasma exchange has been reported to provide clinical benefit for such cases. In thisreport, we present a case of a brown recluse spider bite that was successfully treated with therapeutic plasma exchange and compare it with previous case reports.</p></div>","PeriodicalId":49422,"journal":{"name":"Transfusion and Apheresis Science","volume":"63 4","pages":"Article 103960"},"PeriodicalIF":1.9,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141333153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Analysis of erythrocyte and iron study data among plateletpheresis donors in Hangzhou, China 中国杭州血小板球蛋白捐献者红细胞和铁研究数据分析
IF 1.9 4区 医学 Q4 HEMATOLOGY Pub Date : 2024-06-12 DOI: 10.1016/j.transci.2024.103959
Chunyan Li , Zhongxiu Wu , Beijie Guo , Ruohua Ba , Haiyan Yang , Yinhong Zheng

Background

The purpose of this study is to obtain the iron parameters level of blood donors and the population who need to pay attention to iron parameters level in this area.

Methods

A total of 993 plateletpheresis donors were included in this study, including 798 males and 195 females. The results of erythrocyte and iron parameters of blood donors were compared and analyzed in different groups according to the gender, age and number of blood donations.

Result

The proportion of men and women with low serum ferritin (SF) levels was 10.8 % and 27.7 %, respectively. The mean levels of serum iron (SI), SF, transferrin saturation (Tfs), hemoglobin (Hb) and hematocrit (HCT) of male blood donors decreased with the increase of age groups, but there was no significant statistical difference between the results of female blood donors. The level of SI, SF, Tfs, Hb and HCT of male donors decreased with the increase of blood donations in the past year, while TRF and TIBC increased. The level of Hb, HCT and SF of female donors showed no significant downward trend, while the levels of TRF increased with increasing donations in the past year, excluding first-time donors. The SI of female donors trended down, and TIBC trended up with increasing donations.

Conclusion

Blood collection institutions need to focus on iron parameters levels in older and frequent male donors, and young fertile female donors.

研究背景 本研究的目的是了解献血者的铁指标水平以及需要关注铁指标水平的人群。结果血清铁蛋白(SF)水平偏低的男性和女性比例分别为 10.8%和 27.7%。男性献血者的血清铁(SI)、SF、转铁蛋白饱和度(Tfs)、血红蛋白(Hb)和血细胞比容(HCT)的平均水平随年龄组的增加而降低,但女性献血者的结果无明显统计学差异。男性献血者的 SI、SF、Tfs、Hb 和 HCT 水平随着过去一年献血量的增加而下降,而 TRF 和 TIBC 水平则上升。女性献血者的血红蛋白、血细胞比容和 SF 水平没有明显的下降趋势,而 TRF 水平则随着过去一年献血量的增加而上升,首次献血者除外。女性献血者的 SI 呈下降趋势,而 TIBC 则随着献血量的增加呈上升趋势。
{"title":"Analysis of erythrocyte and iron study data among plateletpheresis donors in Hangzhou, China","authors":"Chunyan Li ,&nbsp;Zhongxiu Wu ,&nbsp;Beijie Guo ,&nbsp;Ruohua Ba ,&nbsp;Haiyan Yang ,&nbsp;Yinhong Zheng","doi":"10.1016/j.transci.2024.103959","DOIUrl":"https://doi.org/10.1016/j.transci.2024.103959","url":null,"abstract":"<div><h3>Background</h3><p>The purpose of this study is to obtain the iron parameters level of blood donors and the population who need to pay attention to iron parameters level in this area.</p></div><div><h3>Methods</h3><p>A total of 993 plateletpheresis donors were included in this study, including 798 males and 195 females. The results of erythrocyte and iron parameters of blood donors were compared and analyzed in different groups according to the gender, age and number of blood donations.</p></div><div><h3>Result</h3><p>The proportion of men and women with low serum ferritin (SF) levels was 10.8 % and 27.7 %, respectively. The mean levels of serum iron (SI), SF, transferrin saturation (Tfs), hemoglobin (Hb) and hematocrit (HCT) of male blood donors decreased with the increase of age groups, but there was no significant statistical difference between the results of female blood donors. The level of SI, SF, Tfs, Hb and HCT of male donors decreased with the increase of blood donations in the past year, while TRF and TIBC increased. The level of Hb, HCT and SF of female donors showed no significant downward trend, while the levels of TRF increased with increasing donations in the past year, excluding first-time donors. The SI of female donors trended down, and TIBC trended up with increasing donations.</p></div><div><h3>Conclusion</h3><p>Blood collection institutions need to focus on iron parameters levels in older and frequent male donors, and young fertile female donors.</p></div>","PeriodicalId":49422,"journal":{"name":"Transfusion and Apheresis Science","volume":"63 4","pages":"Article 103959"},"PeriodicalIF":1.9,"publicationDate":"2024-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1473050224001277/pdfft?md5=37d0a5690248f6addb859143a097fde9&pid=1-s2.0-S1473050224001277-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141324844","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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Transfusion and Apheresis Science
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