Journal of blood disorders & transfusion最新文献

英文中文

Stem cell transplantation: Donors as research objects, the donor search process and beyond 干细胞移植:供体作为研究对象，供体搜索过程及以后

Journal of blood disorders & transfusion

Pub Date : 2019-04-04 DOI: 10.4172/2155-9864-c1-040

S. Morsch

引用次数: 0

Haemophilia a carrier detection among Sudanese families: DNA linkage analysis approaches 血友病携带者检测苏丹家庭:DNA连锁分析方法

Journal of blood disorders & transfusion

Pub Date : 2019-04-04 DOI: 10.4172/2155-9864-C1-041

pRayan Elsheikh, Eltaher Khalilp

引用次数: 0

Heparin Induced Thrombocytopenia in a Patient with Antiphospholipid Syndrome 抗磷脂综合征患者肝素诱导的血小板减少症

Journal of blood disorders & transfusion

Pub Date : 2019-01-01 DOI: 10.4172/2155-9864.1000411

R. Ahmad, S. Chaudhry

Heparin-induced thrombocytopenia (HIT) is a less commonly encountered adverse reaction of heparin characterized by formation of heparin complex with platelet factor 4 due to formation of autoantibody. HIT is reported in about 2% of all patients receiving heparin, out of which 35% develop thrombosis. In Antiphospholoipid syndrome autoantibodies are generated to phospholipid binding proteins which are risk factors for thrombosis and pregnancy complications. In this report we present case of a patient with recurrent venous thromboembolism receiving heparin and was found to develop HIT with coexistence of Antiphospholipid syndrome.

肝素诱发的血小板减少症(HIT)是一种较不常见的肝素不良反应，其特征是由于自身抗体的形成而与血小板因子4形成肝素复合物。据报道，接受肝素治疗的患者中约有2%发生HIT，其中35%发生血栓形成。抗磷脂综合征产生针对磷脂结合蛋白的自身抗体，磷脂结合蛋白是血栓形成和妊娠并发症的危险因素。在这个报告中，我们提出的病例患者复发静脉血栓栓塞接受肝素和发现发展HIT与共存的抗磷脂综合征。

引用次数: 0

Evaluation of Blood Lead Level as a Risk Factor in Children with Autism Spectrum Disorder: A Case Control Study 评估血铅水平作为自闭症谱系障碍儿童的危险因素:一项病例对照研究

Journal of blood disorders & transfusion

Pub Date : 2019-01-01 DOI: 10.4172/2155-9864.1000415

Ali Ms, Begum Ba, Akhter S, N. K, Uddin Ukm, Akter S, Jolly Yn

The present study deals with the Evaluation of Blood lead (Pb) level as a risk factor in Autistic Children and to determine the association between blood lead level (BLL) and Autism Spectrum Disorder (ASD). It was a casecontrol study. Blood samples were collected from both case (25 of 3-16 years) and control (25 of 3-16 years) groups by vein puncture for the determination of blood lead levels using Energy Dispersive X-ray Fluorescence (EDXRF) technique. Predesigned questionnaires were completed for each case and control group by interviewing the parents or care-givers. The present study revealed that there was a significant difference between mean ages of mother at child’s birth in both case and control group. Significantly more children in case group had parents with higher educational levels and came from families with higher socioeconomic status. Significantly more children in ASD group came from urban areas than rural area. The risk of exposure to air pollution in case group was 14 times more than the control group which is represented by the proximity of child’s residence to high traffic roads. History of pica was exclusively present in case group (p value 0.001) indicating that children in ASD group had more exposure to lead than those of control group. The mean blood levels were 44.18 and 29.22 μg/dl for case and control group respectively. In case group 48% of the children had blood lead level ≥ 10 μg/dl compared to 24% in the control group.

本研究探讨了血铅(Pb)水平作为自闭症儿童危险因素的评价，并探讨了血铅(BLL)水平与自闭症谱系障碍(ASD)的关系。这是一项病例对照研究。采用静脉穿刺法采集病例组(3-16岁中的25例)和对照组(3-16岁中的25例)血液样本，采用能量色散x射线荧光(EDXRF)技术测定血铅水平。通过对父母或照顾者的访谈，对每个病例和对照组完成预先设计的问卷。本研究发现，病例组与对照组的产妇平均出生年龄有显著差异。病例组中有更多的孩子的父母教育水平较高，来自社会经济地位较高的家庭。明显更多的自闭症儿童来自城市地区而不是农村地区。病例组暴露于空气污染的风险是对照组的14倍，其代表是儿童住所靠近交通繁忙的道路。病例组仅存在异食癖史(p值0.001)，表明ASD组儿童的铅暴露量高于对照组。病例组和对照组的平均血药浓度分别为44.18和29.22 μg/dl。病例组48%的儿童血铅水平≥10 μg/dl，而对照组为24%。

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引用次数: 0

Some Hematologic Parameters of Blood Donors at the National Blood Transfusion Service (NBTS), Jos, Nigeria 尼日利亚乔斯国家输血中心献血者的一些血液学参数

Journal of blood disorders & transfusion

Pub Date : 2019-01-01 DOI: 10.4172/2155-9864.1000416

Lugos, Okoh Jb, Polit Uy, V. Ny, Ofojekwu Mjn, Nnanna Ou, Damen Jg, Iheanacho Cu, Ntuhun Bd, Damulak Od

Introduction: A blood donor is expected to be a healthy individual who donates his or her blood for the medical treatment of patients. World Health Organisation (WHO) recommends that only those with good health status should be accepted as blood donors. Full blood count is a standard haematology test that evaluates a blood sample for a variety of basic parameters and partly applicable in the general screening of health. Normal haemoglobin level is one of the requirements for blood donor suitability. Normal haemoglobin alone does not connote normalcy of other haematologic variables. The full blood count of blood donors may reveal other blood measurements that may contribute to the better assessment of donors and standardisation of blood donor selection.Aim: This research is aimed to evaluate some haematological parameters of assumingly healthy volunteer blood donors at the NBTS (National Blood Transfusion Service), in Jos, Plateau State.Methods: A total of 102 potential healthy blood donors from Jos City and Du Village participated in the study. We obtained 2.5 ml of venous blood aseptically from each donor into an EDTA container and mixed. The full blood counts of the samples were all analysed. The values gotten were subjected to statistical analysis using the SPSS version 23 software.Results: The packed cell volume (PCV), total and differential white blood cell counts and platelet count were significantly different compared to local reference ranges. Further, evaluation of the parameters between genders, locations, age groups and occupations of donors, the platelet, PCV and eosinophil counts differed significantly (p=0.042, 0.00 and 0.029 respectively). The average white blood cells (WBC) count was lower among donors in the rural area (p=0.000).Conclusion: There may be a significant number of apparently healthy blood donors with abnormal haematologic parameters. Full blood count should be included in evaluating blood donors to ensure blood and donor safety.

献血者应该是一个健康的人，为病人的医疗而献血。世界卫生组织(WHO)建议，只有健康状况良好的人才应被接受为献血者。全血细胞计数是一种标准的血液学测试，用于评估血液样本的各种基本参数，部分适用于一般健康筛查。正常血红蛋白水平是献血者适宜性的要求之一。血红蛋白正常并不意味着其他血液学指标正常。献血者的全血细胞计数可以揭示其他血液测量，有助于更好地评估献血者和标准化献血者的选择。目的:本研究旨在评估高原州乔斯国家输血服务中心(NBTS)假定健康的志愿献血者的一些血液学参数。方法:对来自乔斯市和杜村的102名潜在健康献血者进行研究。我们从每个供者无菌处取2.5 ml静脉血，放入EDTA容器中混合。所有样本的全血细胞计数都进行了分析。所得数据采用SPSS 23版软件进行统计分析。结果:与当地参考值比较，两组患者的堆积细胞体积(PCV)、白细胞总数、白细胞差异数、血小板计数均有显著性差异。不同性别、不同地区、不同年龄、不同职业的献血者血小板、PCV和嗜酸性粒细胞的评价差异有统计学意义(p分别为0.042、0.00和0.029)。农村献血者的平均白细胞计数较低(p=0.000)。结论:可能有大量表面健康的献血者血液学参数异常。在评估献血者时应包括全血细胞计数，以确保血液和献血者安全。

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引用次数: 5

Prevalence/Incidence of Hereditary and Acquired Thrombophilia Markers among Egyptian Females with Recurrent Pregnancy Loss or IVF Failure 埃及女性复发性流产或体外受精失败的遗传和获得性血栓标志物的患病率/发病率

Journal of blood disorders & transfusion

Pub Date : 2019-01-01 DOI: 10.4172/2155-9864.1000412

A. V., Issa H, R. A

引用次数: 0

A Rare Presentation of ph Chromosome: idic der (22q11) in Blast Crisis Chronic Myeloid Leukemia 细胞危象慢性髓系白血病中罕见的ph染色体:idic der (22q11)

Journal of blood disorders & transfusion

Pub Date : 2019-01-01 DOI: 10.4172/2155-9864.1000414

P. Kumari, M. Mangalagowri, Kavitha Bl, C. ObulaReddy, S. Shanthala, M. Mahadavaprasad, Madhumathi Ds, Govinda Bk

The ider(22)t(9;22)(q34;q11) is a rare secondary karyotypic abnormality of Ph chromosome positive chronic myeloid leukemia associated with disease progression, poor clinical outcome and short survival in most of the previously reported cases. The present case of CML with idic der(22)t(9;22)(q34;q11) or idic der Ph with hybrid transcript ratio of BCR-ABL being 97% showed a complex karyotype: 48,XY,+8,t(9;22)(q34;q11)i dic(22)(q11),+idic der(22)t(9;22)(q34;q11). On treatment with Imatinib the initial transcript ratio was reduced to 12.125%, 0.932% but later increased to 93%. The patient developed extra medullary myeloid cell tumor of testis after a year of treatment followed by the detection of T3151 mutation. Cytogenetics provides an evidence for progression of disease at an earlier phase than other markers in this case.

ider(22)t(9;22)(q34;q11)是一种罕见的Ph染色体阳性慢性髓性白血病继发性核型异常，在大多数先前报道的病例中，与疾病进展、临床预后差和生存期短有关。本例CML伴idic der(22)t(9;22)(q34;q11)或idic der Ph, BCR-ABL杂交转录比为97%，显示复杂核型:48,XY，+8,t(9;22)(q34;q11)i dic(22)(q11)，+idic der(22)t(9;22)(q34;q11)。伊马替尼组初始转录率分别降至12.125%、0.932%，后升高至93%。患者在治疗一年后出现睾丸髓外髓样细胞瘤，随后检测到T3151突变。在这种情况下，细胞遗传学提供了比其他标志物更早阶段疾病进展的证据。

引用次数: 0

CALR and CD47: An Insight into Their Roles in the Disease Progression of MDS and MPN CALR和CD47在MDS和MPN疾病进展中的作用

Journal of blood disorders & transfusion

Pub Date : 2018-11-20 DOI: 10.4172/2155-9864.1000413

K. Boasman, M. Simmonds, C. Rinaldi

Myelodysplastic syndrome and myeloproliferative neoplasms are clonal myeloid disorders arising from haematopoietic stem cells that have the tendency to progress into acute myeloid leukaemia. Multiple prognostic scoring systems have been proposed and utilised in clinical practice to predict disease evolution, however none of them can predict treatment response. In solid tumours, the relationship between the pro-phagocytic calreticulin and the anti-phagocytic CD47 is repeatedly investigated. Overexpression of calreticulin has been documented to produce a pro-phagocytic signal in solid tumour and it is often counteracted by a concomitant expression of the anti-phagocytic CD47 as they act in response to one another, reflecting an apoptosis vs survival mechanism in response to chemotherapy. The role of both calreticulin and CD47 are currently poorly understood in myeloid malignancies including myelodysplastic syndrome and myeloproliferative neoplasms. The aim of this review is to elaborate on the current understanding round the roles and implications of calreticulin and CD47 signalling with in solid and haematological cancers, discuss potential roles for calreticulin and CD47 expression in transformation of myeloid cells in patients with MDS or MPN into AML and how these advances are starting to be used to design new therapeutic strategies to determine disease progression and treatment response in both solid cancer and myeloid malignancies.

骨髓增生异常综合征和骨髓增生性肿瘤是由造血干细胞引起的克隆性骨髓疾病，有发展为急性髓细胞白血病的趋势。多种预后评分系统已被提出并在临床实践中用于预测疾病演变，但它们都无法预测治疗反应。在实体瘤中，反复研究促吞噬细胞的钙网蛋白和抗吞噬细胞的CD47之间的关系。钙网织蛋白的过表达已被证明在实体瘤中产生促吞噬信号，并且当它们相互反应时，它通常被伴随的抗吞噬CD47的表达所抵消，反映了对化疗反应的细胞凋亡与生存机制。钙网织蛋白和CD47在骨髓恶性肿瘤（包括骨髓增生异常综合征和骨髓增生性肿瘤）中的作用目前尚不清楚。本综述的目的是阐述目前对钙网织蛋白和CD47信号传导在实体癌和血液学癌症中的作用和意义的理解，讨论钙网蛋白和CD47表达在MDS或MPN患者髓系细胞转化为AML中的潜在作用，以及这些进展如何开始用于设计新的治疗策略，以确定癌症和髓系恶性肿瘤的疾病进展和治疗反应。

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引用次数: 3

Binding of Factor VIII to Lipid Nanodiscs Increases its Clotting Function in a Mouse Model of Hemophilia A. 在血友病a小鼠模型中，因子VIII与脂质纳米盘的结合增加了其凝血功能。

Journal of blood disorders & transfusion

Pub Date : 2015-12-01 DOI: 10.4172/2155-9864.1000325

Keri Csencsits-Smith, Krill Grushin, Svetla Stoilova-McPhie

Background: Hemophilia A is a congenital bleeding disorder caused by defective or deficient factor VIII (FVIII). The active form of FVIII is the co-factor for the serine protease factor IXa (FIXa) in the membrane-bound intrinsic tenase (FVIIIa-FIXa) complex. The assembly of the FVIIIa-FIXa complex on the activated platelet surface is critical for successful blood clotting.

Objectives: To characterize the role of lipid nanodiscs (ND) for on FVIII function in vivo and test the lipid ND as a delivery system for FVIII. To evaluate the potential of binding recombinant FVIII to ND as improved treatment for Hemophilia A.

Methods: Recombinant porcine FVIII (rpFVIII) was expressed and characterized in solution, and when bound to ND. The rpFVIII, ND and rpFVIII-ND complexes were characterized via transmission electron microscopy. Functional studies were carried out using aPTT tests and time resolved tail snip studies of hemophilic mice.

Results: Functional rpFVIII was successfully assembled on lipid ND. When injected in hemophilic mice, the rpFVIII-ND complexes showed a pronounced pro-coagulant effect, which was stronger than that of rpFVIII alone. While injection of the ND alone showed a pro-coagulant effect this effect was not additive, implying that the rpFVIII-ND complexes have a synergistic effect on the clotting process in hemophilic mice.

Conclusions: Binding of rpFVIII to ND prior to its injection in hemophilic mice significantly improves the therapeutic function of the protein. This represents a meaningful step towards a new approach to modulate blood coagulation at the membrane-bound FVIII level and the assembly of the intrinsic tenase complex.

背景:A型血友病是一种由因子VIII (FVIII)缺陷引起的先天性出血性疾病。FVIII的活性形式是膜结合内张力酶(fviia -FIXa)复合体中丝氨酸蛋白酶因子IXa (FIXa)的辅因子。fviia - fixa复合物在活化血小板表面的组装是成功凝血的关键。目的:表征脂质纳米片(ND)在体内对FVIII功能的作用，并测试脂质纳米片作为FVIII的递送系统。方法:采用重组猪FVIII蛋白(rpFVIII)在溶液中和与ND结合时进行表达和表征。通过透射电镜对rpFVIII、ND和rpFVIII-ND配合物进行了表征。采用aPTT试验和时间分辨尾剪对血友病小鼠进行功能研究。结果:功能性rpFVIII成功组装在脂质ND上。当注射到血友病小鼠体内时，rpFVIII- nd复合物显示出明显的促凝作用，比单独注射rpFVIII更强。虽然单独注射ND显示促凝作用，但这种作用不是叠加性的，这意味着rpFVIII-ND复合物对血友病小鼠的凝血过程具有协同作用。结论:在血友病小鼠注射前，将rpFVIII与ND结合可显著提高该蛋白的治疗功能。这是朝着在膜结合FVIII水平上调节血液凝固和内在张力酶复合物组装的新方法迈出的有意义的一步。

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引用次数: 6

Characterization of Bone Marrow-Derived Dendritic Cells Developed in Serum-Free Media and their Ability to Prevent Type 1 Diabetes in Nonobese Diabetic Mice 无血清培养基中培养的骨髓来源树突状细胞的特性及其在非肥胖糖尿病小鼠中预防1型糖尿病的能力

Journal of blood disorders & transfusion

Pub Date : 2014-03-22 DOI: 10.4172/2155-9864.1000206

Benjamin M. Looney, Anna V. Chernatynskaya, M. Clare-Salzler, C. Xia

Dendritic cells (DC) have been investigated as a cell-based therapy for Type 1 Diabetes (T1D). BM-DC expanded ex vivo with GM-CSF and IL-4 is typically cultured with fetal bovine serum (FBS). The effect of FBS on NOD BM-DC has not been extensively studied. In the present study we compare BM-DC generated in serum-free culture media (X-VIVO20; FBS−) with BM-DC generated in media containing 10% FBS (RPMI1640/10%FBS; FBS+). We show that FBS− BM-DC display a phenotype and cytokine-producing profile distinct from FBS+ BMDC. Additionally, compared to FBS+ BM-DC, we show evidence of an altered Th cell response induced by FBS− BM-DC. Finally, we demonstrate that only FBS− BM-DC prevent the onset of T1D and induce increased levels of CD4+Foxp3+ regulatory T cells as well as a long-lasting β cell-specific T cell response. This study indicates that serum-free media generates a more tolerogenic BM-DC capable of preventing T1D in the NOD mice.

树突状细胞(DC)作为一种基于细胞的治疗1型糖尿病(T1D)的方法已被研究。用GM-CSF和IL-4体外扩增的BM-DC通常与胎牛血清(FBS)一起培养。FBS对NOD BM-DC的影响尚未得到广泛研究。在本研究中，我们比较了无血清培养基(X-VIVO20;FBS−)，在含有10%FBS的培养基中生成BM-DC (RPMI1640/10%FBS;的边后卫+)。我们发现FBS - BM-DC表现出与FBS+ BMDC不同的表型和细胞因子产生谱。此外，与FBS+ BM-DC相比，我们发现FBS - BM-DC诱导的Th细胞反应发生了改变。最后，我们证明了只有FBS - BM-DC可以预防T1D的发生，并诱导CD4+Foxp3+调节性T细胞水平的增加，以及持久的β细胞特异性T细胞反应。本研究表明，无血清培养基可在NOD小鼠中产生耐受性更强的BM-DC，能够预防T1D。

引用次数: 5

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