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BACKGROUND: Multi-transfused thalassemic children are at higher risk of acquiring transfusion-transmitted infections (TTIs). There are limited data available on TTIs among thalassemic children, especially on its impact on their quality of life (QoL). AIM: The aim of this study is to find out the proportion of multi-transfused β-thalassemia major (β-TM) children suffering from TTIs, its risk factors and impact on QoL. METHODS: This was a hospital-based, analytical observational study, cross-sectional in design, conducted among 328 β-TM children and their caregivers attending thalassemia day care unit of a medical college during May 2015-April 2016, with a structured schedule. Data were analyzed with appropriate statistical methods using the Statistical Package for the Social Sciences. RESULTS: Two-fifth (39.9%) of them were found to have TTIs with hepatitis C being the most common (34.5%), followed by hepatitis B (4.5%) and human immunodeficiency virus (1.8%). In the multivariable model, place of residence (adjusted odds ratio [AOR] - 2.23 [1.19-4.17]), per capita monthly family income (AOR - 1.84 [1.10-3.07]), and blood transfusion frequency (AOR - 1.19 [1.10-1.29]) were significant predictors of TTIs adjusted with their age, age at diagnosis, last pretransfusional hemoglobin level, size of spleen, and caregivers knowledge regarding the disease. The study participants with TTIs had a lower QoL compared to others as there were significant differences in between the total QoL scores ([49.9 ± 15.6 vs. 57.4 ± 15.5], P ≤ 0.001) and its various domains. CONCLUSION: There was high burden of TTIs among multi-transfused β-TM children and it has significant negative impact on their quality of lives.

In a prospective study, 254 of 5649 unselected patients scheduled for surgery at our hospital were identified preoperatively as having either acquired (n=182) or inherited (n=72) impaired primary hemostasis (platelet dysfunction including von Willebrand disease). All patients were initially pretreated with desmopressin (DDAVP). Response to DDAVP or subsequent treatment(s) was defined as correction of any one of the abnormal PFA-100 platelet function tests. The non-responders were additionally treated with tranexamic acid or aprotinin; those with von Willebrand disease (vWD) received factor VIII concentrates with von Willebrand factor (vWF). Those still unresponsive to therapy received conjugated estrogens and, as a last attempt, a platelet transfusion. The administration of DDAVP led to a correction of platelet dysfunction in 229 of the 254 patients treated (90.2%). Tranexamic acid was effective in 12 of 16, aprotinin in 3 of 5, and factor VIII concentrates with vWF in all 4 patients with unresponsive to DDAVP. The remaining 6 patients were pretreated with conjugated estrogens, and 2 of these patients were additionally treated with platelet transfusion. The frequency of blood transfusion was lower, but not statistically significant (9.4% vs. 12.2%: p = 0.202) in preoperatively treated patients with impaired hemostasis than in patients without impaired hemostasis. In a retrospective group, the frequency of blood transfusion was statistically significant higher (89.3% vs. 11.3%: p < 0.001) in patients without preoperative correction of impaired hemostasis than in patients without impaired hemostasis. Preoperative correction of impaired primary hemostasis is possible in nearly all patients affected, and results in a reduction of homologous blood transfusions.

Abstract Introduction/Objective Karl Landsteiner discovered the ABO blood group system in 1900, which was the first human blood group system. The Rh blood group system was found forty years later. The antigens on the surface of red blood cells are used to classify people into ABO blood groups. In the ABO system, there are four types: A, B, AB, and O; in the rhesus system, there are two types: Rh-positive and Rh-negative. Blood group identification is critical for efficiently managing blood banks and blood transfusion services to avoid serious transfusion reactions. Methods/Case Report The study was performed on a total of 5996 healthy blood donors in the United Arab of Emirates (UAE). ABO and Rh (D) groupings were performed on all donors' samples by the gel electrophoresis technique. Data on the frequency of ABO and Rh(D) blood groups were reported in numbers and percentages. Results (if a Case Study enter NA) The study revealed that type O is the most prevalent blood group in the United Arab Emirates (43.83%), followed by A at 26.68% and B at 23.90%, with AB having the lowest prevalence at 5.58%; O > A > B > AB. Our study found that 92.57% of the donor population was Rh-positive, whereas 7.43% was Rh- negative. The IA, IB, and IO allele frequencies were determined using the Hardy-Weinberg rule of equilibrium. The gene frequencies are calculated to be 0.1942 for IA (p), 0.1438 for IB (q), and 0.6620 for IO (r). O (r) has the most value in UAE, followed by A (p) and B (q); O > A > B. OO constituted 43.83% of the homozygous types, AA 3.77%, and BB 2.06%. The heterozygous types were 25.71% for AO, 19.03% for BO, and 5.58% for AB. Conclusion The study gives reliable ABO gene frequency statistics and information on the distribution of ABO blood group Rh groups of various alleles in the United Arab Emirates. This knowledge aids in the efficient management of the blood bank's inventory. It will aid transfusion services in anticipating future health issues and improving blood transfusion practice. The study is the first to give reliable ABO gene frequency statistics and information on the distribution of ABO blood group Rh groups of various alleles in the United Arab Emirates. This knowledge aids in the efficient management of the blood bank's inventory. It will aid transfusion services in anticipating future health issues and improving blood transfusion practice.

Background: ABO–rhesus (Rh) blood testing screens blood types according to the antigenic properties of red blood cells. Objective: This study reports the allelic and phenotypic frequency distribution of the ABO and Rh blood groups in pregnant women who attended antenatal care (ANC) at Zewditu Memorial Hospital in Addis Ababa, Ethiopia, and the likelihood for the occurrence of erythroblastosis fetalis (E. fetalis). Methods: A retrospective study was conducted on pregnant women who attended ANC from 2015 to 2019 and typed for ABO and Rh blood groups. The Hardy–Weinberg equilibrium was used to determine the allelic frequency of ABO and Rh blood types. The likelihood of the occurrence of E. fetalis was computed. Results: Among the 2453 women who had been admitted to ANC, 2407 (98.1%) pregnant women who had been typed for the ABO and Rh blood groups were included in this retrospective study. We found that Type O blood was the most common one (38.9%), while Types A (31.3%), B (23.8%), and AB (6.0%) blood were scored with modest to lower proportions. Among blood group–typed women, 94.2% were Rh‐positive. The allelic frequency of O was 0.62, whereas A (0.22) and B (0.16) had modest proportions. The allelic frequency of D was 0.76 and d was 0.24. The likelihood of the occurrence of E. fetalis was 5%. Our findings show that both the ABO ( χ ‐ s q u a r e d = 6.1439, d f = 3, p v a l u e = 0.1048) and the Rh ( χ ‐ s q u a r e d = 0.000103, d f = 1, p v a l u e = 0.9919) blood groups were segregated at the Hardy–Weinberg proportions. Studies need to investigate the evolutionary forces that have made the ABO and Rh blood types segregate at the Hardy–Weinberg proportion.

To investigate the expression and distribution of Rh phenotypes (C, c, D, E, e) among voluntary blood donors in a specific region of East China, to establish a regional Rh phenotype database, and to enhance the precision and efficacy of clinical blood transfusions. A total of 28979 blood samples were collected from voluntary donors at a central blood station in East China between May 2023 and December 2023. An automated blood type analyzer was used to determine Rh phenotypes, which were then applied clinically for ABO and Rh blood type-matching in transfusions. Analysis of 28672 RhD-positive donors identified 13 RhD variants and eight Rh phenotypes, with the most common being CCee (42.69%) and CcEe (35.27%). Antigen frequencies were e (92.07%), C (87.85%), c (56.75%), and E (47.65%). Among 307 RhD-negative donors, seven Rh phenotypes were identified, with ccee (60.26%) and Ccee (29.32%) being the predominant ones. Antigen frequencies were e (99.67%), c (96.09%), C (34.53%), and E (6.84%). These findings supported 1834 ABO- and Rh- blood type-matching transfusions, but no significant difference was observed between ABO-compatible and dual-system compatible transfusions ( > 0.05). Additionally, it was found that there are significant differences compared to populations from India and other regions ( > 0.05). In this region of East China, the prevalence of RhD variants among voluntary blood donors was 0.045%. The predominant Rh phenotypes were CCDee and CcDEe, with the highest frequencies observed for the e and C antigens. And the frequency of Rh phenotypes in this region differs from related studies in other areas. It is essential to strengthen the establishment of a rare blood type database in East China to provide data support for clinical compatible blood transfusion.

The sporadic nature of blood transfusion therapy coupled with the alteration of HAMP genes may exacerbate the risk of iron burden in sickle cell anaemia (SCA) patients. The study determined the polymorphic distribution of the HAMP promoter gene rs10421768 and hepcidin levels in SCA patients. Sixty participants aged ≥12years [45 SCA patients and 15 controls (HbA)] were recruited from 15th March, 2023 to 20th July, 2023 for a case-control study at Methodist Hospital Wenchi, Ghana. Complete blood count and hepcidin levels assessment were done using haematology analyzer and ELISA, respectively. Genomic DNA was extracted using the Qiagen Kit, and HAMP gene rs10421768 (c.-582 A>G) was sequenced using the MassARRAY method. Data were analysed using SPSS version 26.0. The frequencies of the HAMP promoter rs10421768 genotypes AA, AG, and GG were 64.4%, 33.3%, and 2.2% in SCA patients, and 86.7%, 13.3%, and 0% in the controls, respectively. Serum hepcidin levels were significantly higher among controls than cases [204.0 (154.1-219.3) vs 150.2 (108.1-195.6)μg/L, p<0.010]. Participants with HAMP rs10421768 homozygous A genotype had higher serum levels of hepcidin compared with those in the wild genotypes (AG/GG) group [(188.7 (130.9-226.9) vs 136.8 (109.7-157.8)μg/L, p<0.016]. Disease severity and blood cell parameters were not associated with the HAMP variants (p>0.05). The HAMP promoter rs10421768 AA genotype has the highest frequency of distribution and the GG genotype with the least distribution. Participants with HAMP rs10421768 G allele (c.-582A>G) had reduced levels of hepcidin. HAMP rs10421768 genotypes had no association with blood cell parameters and disease severity. The HAMP rs10421768 genotypes may influence serum levels of hepcidin. Further study is required to elucidate the potential effect of the G allele on hepcidin transcription.

Clonal hematopoiesis of indeterminate potential (CHIP) occurs in an age-related manner and associates with an increased risk of hematologic cancer, atherosclerotic disease, and shorter overall survival. Little is known about the cell of origin, repartition patterns of clonal mutations within the hematopoietic differentiation tree, and its dynamics under evolutionary pressure. Using targeted sequencing, CHIP was identified in 121 out of 437 elderly individuals (27.7%). Variant allele frequencies (VAFs) of 91 mutations were studied in six peripheral blood cell fractions. VAFs were significantly higher in monocytes, granulocytes, and NK-cells compared to B- or T cells. In all cases with available bone marrow material, mutations could be identified in Lin−CD34+CD38− HSCs with subsequent expansion to myeloid primed progenitors. In 22 patients with solid cancer receiving (radio-)chemotherapy, longitudinal study of 32 mutations at 121 time points identified relative VAF changes of at least 50% in 13/32 mutations. VAFs of DNMT3A, were stable in 12/13 cases (P < .001). Cancer patients with a clonal mutation other than DNMT3A required more often red blood cell transfusions and dose reductions. Our results provide novel insights into cellular distribution of clonal mutations, their dynamics under chemotherapy, and advocate for systematic analyses for CHIP in cancer patients.

The present study aimed to report our real-life experience of the TPO receptor agonist lusutrombopag for cirrhotic patients with low platelet counts. We studied platelet counts in 1,760 cirrhotic patients undergoing invasive procedures at our hospital between January 2014 and December 2017. In addition, we studied 25 patients who were administered lusutrombopag before invasive procedures between June 2017 and January 2018. Effectiveness of lusutrombopag to raise platelet counts and to avoid transfusion and treatment-related adverse events were analyzed. In 1,760 cirrhotic patients without lusutrombopag prior to invasive procedures, proportion of patients whose platelet counts <50,000/μL and needed platelet transfusions were 66% (n = 27/41) for radiofrequency ablation, 43% (n = 21/49) for transarterial chemoembolization, and 55% (n = 21/38) for endoscopic injection sclerotherapy / endoscopic variceal ligation, respectively. In 25 cirrhotic patients treated by lusutrombopag prior to the invasive procedures, platelet counts significantly increased compared with baseline (82,000 ± 26,000 vs. 41,000 ± 11,000/μL) (p < 0.01). Out of 25 patients, only 4 patients (16%) needed platelet transfusion before the invasive procedures. The proportion of patients with low platelet count and who needed platelet transfusions was significantly low in patients treated with lusutrombopag compared to those not treated with lusutrombopag (16% (4/25) vs. 54% (69/128), p = 0.001). Platelet counts after lusutrombopag treatment and prior to invasive procedures were lower in patients with a baseline platelet count ≤30,000/μL (n = 8) compared with those with a baseline platelet count >30,000/μL (n = 17) (50,000 ± 20,000 vs 86,000 ± 26,000/μL, p = 0.002). Patients with a baseline platelet count ≤30,000/μL with spleen index (calculated by multiplying the transverse diameter by the vertical diameter measured by ultrasonography) ≥40 cm2 (n = 3) had a lower response rate to lusutrombopag compared to those with spleen index <40 cm2 (n = 5) (0% vs. 100%, p = 0.02). Hemorrhagic complication was not observed. Recurrence of portal thrombosis was observed and thrombolysis therapy was required in one patient who had prior history of thrombosis. Lusutrombopag is an effective and safe drug for thrombocytopenia in cirrhotic patients, and can reduce the frequency of platelet transfusions.

African populations are characterized by high degree of genetic diversity. This high genetic diversity could result from the natural selection pressure. Several studies have described an association between some genetic diversities and difference of susceptibility to infectious diseases like malaria. It seems therefore important to consider genetic diversity impact when interpreting results of clinical trials in malaria endemic areas. This study aimed to determine the genetic polymorphism with erythrocyte traits in different populations of malaria endemic area in Mali. The cross-sectional surveys were carried out in different ethnic groups living in malaria endemic areas in Mali. Six milliliters of whole blood were collected in EDTA vials from each participant after informed consent has been obtained. The ABO, RH, Kell, MNSs, Kidd and Duffy systems phenotypes were assessed by the technique of gel filtration. A total of 231 subjects were included from six villages. The blood groups phenotypes O (40.7%) and A (31.2%) were more frequent with respective allele frequencies of 0.65 and 0.21. In the RH system the haplotypes R0 (0.55), r (0.20) and R1 (0.13) were the most frequent. Seven percent (7%) of Duffy positive and 4% of Glycophorin B deficiency (S-s-) were observed among participants. All participants were Kell negative. ABO and RH systems were polymorphic in these ethnic groups in Mali. Their implication in susceptibility to malaria should be taken into account in clinical trials interpretation, and for prevention of blood transfusion risks during anemia frequently caused by malaria in children.

Blood group antigens, which are prominently expressed in red blood cells, are important in transfusion medicine. The advent of high-throughput genome sequencing technology has facilitated the prediction of blood group antigen phenotypes based on genomic data. In this study, we analyzed data from a large Korean population to provide an updated prevalence of blood group antigen phenotypes, including rare ones. A robust dataset comprising 72,291 single nucleotide polymorphism arrays, 5318 whole-exome sequences, and 4793 whole-genome sequences was extracted from the Korean Genome and Epidemiology Study, Genome Aggregation Database, and Korean Variant Archive and then analyzed. The phenotype prevalence of clinically significant blood group antigens, including MNSs, RHCE, Kidd, Duffy, and Diego, was predicted through genotype analysis and corroborated the existing literature. We identified individuals with rare phenotypes, including 369 (0.51%) with Fy(a−b+), 188 (0.26%) with Di(a+b−), and 16 (0.02%) with Jr(a−). Furthermore, we calculated the frequencies of individuals with extremely rare phenotypes, such as p (0.000004%), Kell-null (0.000310%), and Jk(a−b−) (0.000438%), based on allele frequency predictions. These findings offer valuable insights into the distribution of blood group antigens in the Korean population and have significant implications for enhancing the safety and efficiency of blood transfusion.