Blood Transfusion Therapy Research Articles

Characterization of Common β-Thalassemia Major Mutations in Southwest Iran with Respect to Biochemical Parameters, Oxidative Status and Complications

Background & Objective: Beta-thalassemia are among the most common autosomal recessive genetic disorders in Iran, especially in Khuzestan province. Beta-thalassemia exhibits significant phenotype heterogeneity and there are currently more than 200 known mutations in this region. Oxidative stress exacerbates multiple disorders, including thalassemia, an inherited hemolytic anemia caused by globin gene mutations. We aim to characterize significant mutations of widespread β-thalassemia in south-western Iran with respect to biochemical parameters, oxidative status and complications of diseases. Material & method: Forty-five patients, aged between 15-35 years with β-thalassemia major were selected. The patients were receiving regular blood transfusion and chelation therapy and have been previously characterized to bear beta globin gene mutations. The subjects’ medical histories were documented by review of previous medical records. We also determined biochemical parameters including glycemic and iron indices, hepatic and renal function tests, oxidative stress markers and levels of advanced glycation end product species (Carboxy methyl lysine and Pentosidin). Results: The most common mutation was found to be CD36/37(28.9%) followed by IVSII-1, and IVSI-110. Values of iron indices were significantly different in various mutation groups. Carboxy methyl lysine and pentosidine were found to be higher in the β-thalassemia patients with IVSII-1 and IVSI-110, respectively. Also sLOX-1 was found to be significantly higher in IVSI-110 group. Complications of the disease were differently presented in mutation groups and hemochromatosis, hepatomegaly, and diabetes were among the most common problems. Conclusion: About 72 % of β-thalassemia major cases in southwest Iran result form 3 common mutations with different clinical and laboratory presentations. Molecular genetic testing can be helpful to evaluate the patients’ situation.

Study on Management of Blood Transfusion Therapy in Patients with Hereditary Spherocytosis.

Hereditary spherocytosis (HS) is a chronic hemolytic disorder caused by inherited defects in the red blood cell membrane. This study discusses the treatment strategy for the decline in hemoglobin level in three HS probands with moderately severe or severe hemolysis and summarizes the appropriate laboratory tests that help improve clinical management of blood transfusion in HS patients. Three probands who were diagnosed with HS in our hospital and their family members were included in this study. Clinical data of the three families were reviewed to summarize their hematopoietic characteristics. DNA from all family members of the 3 HS probands was amplified by polymerase chain reaction (PCR) and sequenced by the Sanger method to assess genetic relation for HS. Based on the sequencing results, the type of mutated membrane protein in each proband was analyzed using the eosin-5′-maleimide (EMA) binding test and sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE). The hemoglobin level was reduced in all 3 probands after different levels of infection. The fluorescence of EMA-labeled red blood cell (RBC) was decreased. DNA sequencing showed that His54Pro, Leu1858Val, and 6531-12C>T compound heterozygous mutations were present in the SPTA1 gene of patient I-1, Arg344Gln and c.609+86G>A heterozygous mutations were present in the SLC4A1 gene of patient II-1, and Leu2032Pro homozygous mutation was present in the SPTB gene of patient III-1. SDS-PAGE results demonstrated that the concentration of band 3 was reduced in II-1, whereas the levels of the corresponding mutant proteins in the other probands were unchanged. The family members of the respective patients presented mutations in major genes causing HS. The Leu2032Pro mutation identified in patient III-1 is a new missense mutation of the SPTB gene in the Chinese population that has never been reported in literature previously. The presence or absence of acute or chronic infections is a critical deciding factor for the treatment and clinical management of HS patient via blood transfusion. For patients with infections, hemoglobin concentration can be restored once the infection is controlled, thus obviating the need for proper infection control before blood transfusion.

Assessment of thyroid function status in transfusion dependent thalassemic children

Background: Frequent blood transfusions have been associated with serum iron overload, which may result in hypothyroidism and other endocrine abnormalities in transfusion dependent thalassemic patients. Thyroid dysfunction has been reported in a number of studies on these pts. The aim of the study is to assess the thyroid function status among the transfusion dependent thalassemic children.Methods: This was a cross sectional study conducted in the department of transfusion medicine, department of Pediatric hematology and oncology and department of hematology, Bangabandhu Sheikh Mujib medical university (BSMMU), Dhaka for a period of 18 months. A total number of 86 children with transfusion dependent thalassemia who met the inclusion and exclusion criteria were studied. Demographic data as well as history of blood transfusion and chelation therapy were collected. Thyroid function and iron load status were evaluated by measuring serum FT4, TSH and ferritin levels.Results: Out of 86 thalassemia patients, euthyroid patients were 77 (89.5%) and hypothyroid patients were 9 (10.5%). Eight (9.3%) patients were compensated and 1 (1.2%) was uncompensated hypothyroidism. Statistically significant association was found between number of blood transfusion and serum ferritin level (≥2000 ng/ml) with development of hypothyroidism in thalassemic patients. Mean ferritin level was higher in hypothyroid (4797.8±2027.4 ng/ml) group than in euthyroid (3628.8±2448.5 ng/ml) groups.Conclusions: Frequency of hypothyroidism was 10.5% (compensated 9.3% and uncompensated 1.2%). Number of blood transfusion and ferritin level were found significantly associated with the development of hypothyroidism in thalassemic patients.

An Overview of Therapy Guidelines for Cardiac Arrest and the Potential Benefits of Hemoglobin-Based Oxygen Carriers

Currently, there is an unmet therapeutic need for the medical management of cardiac arrest, as is evident from the high mortality rate associated with this condition. These dire outcomes can be attributed to the severe nature and poor prognosis of this disorder. However, the current treatment modalities, while helping to augment survival, are limited and do not offer adequate improvements to outcomes. Treatment modalities are particularly lacking when considering the underlying pathophysiology of the metabolic phase of cardiac arrest. In this study, we explore the three phases of cardiac arrest and assess the factors related to positive clinical outcomes and survival for these events. Furthermore, we evaluate the present guidelines for resuscitation and recovery, the issues related to ischemia and tissue reperfusion, and the benefit of oxygen-delivery therapeutic methods including blood transfusion therapy and synthetic hemoglobins (HBOCs). The current therapy protocols are limited specifically by the lack of an efficient method of oxygen delivery to address the metabolic phase of cardiac arrest. In this article, we investigate the next generation of HBOCs and review their properties that make them attractive for their potential application in the treatment of cardiac arrest. These products may be a viable solution to address complications associated with ischemia, reperfusion injury, and organ damage.

CHARACTERISTICS OF BLOOD DONORS, ACCORDING TO THE RESULTS OF COMPLEX LABORATORY TESTS OF PERIFERAL BLOOD

Nowadays, there is a significant storage of donated blood in the world. According to the recommendations of the WHO, adequate and reliable supply of save donated blood can only by carried out on the base of regular voluntary non-paid donations. This particular category of donations in the safest from the point of view of the prospect of having infectious diseases that can be transmitted through blood. Recrutitg and retaining the donors who make voluntary non-paid donations is a priority for the blood service on the way to achieving 100% voluntary non-paid donation of blood and its components. Blood transfusion service and its social component – donor ship must be the priority areas of the state policy because the results of its work are of paramount importance. The main task of the blood transfusion service is supply of high quality components for blood transfusion therapy. Quality of blood components is compliance of properties and specifications of the blood component supplied to the recipient with the set standards. Strict order of conformance with the approved regulations and procedures is important at all technological states and is a cornerstone of blood transfusion service products quality. All actions, planned and implemented, starting with planning donor ship and ending with the finished product manufacturing and storage conditions, are important for ensuring the quality as the final result.

Three cases of alloimmune mediated pancytopenia in calves resembling bovine neonatal pancytopenia

BackgroundBetween 2007 and 2011 several thousands of calves died from bovine neonatal pancytopenia (BNP), a bleeding syndrome triggered by vaccine induced alloantibodies from the dams. Following withdrawal of the involved bovine viral diarrhoea virus (BVDv) vaccine, the incidence of this condition rapidly decreased, with no reported cases in the last 5 years. Here, we report a recent immune-mediated pancytopenia in three calves from two different suckler herds, clinically indistinguishable from BNP.Case presentationThree Belgian Blue suckler calves from two different farms, aged around two weeks, showed multiple bleedings disseminated on the skin and petechiae and ecchymoses on the mucosae. Blood examination confirmed anaemia, leukopenia and thrombocytopenia. BVDv infection was excluded. Despite blood transfusion and cortisone therapy, all three animals died. Necropsy and histology confirmed bone marrow depletion. Binding of IgG from the dams on leukocytes of the calves was demonstrated by flow cytometry. Two calves, originating from the same farm, received colostrum from the same dam. None of the calves were given colostrum replacers or colostrum supplements. No link with the BNP causing BVDv vaccine could be evidenced. However, dams had been vaccinated against bovine herpesvirus 1, parainfluenza-3 virus, bovine respiratory syncytial virus and bluetongue virus serotype 8.ConclusionsAlloimmune mediated pancytopenia was evidenced in three animals, clinically and pathologically indistinguishable from BNP. Whether this disease is again vaccine mediated remains to be determined.

POSC156 Clinical Burden, Treatment Pathway, and Healthcare Resource Utilization of Transfusion-Dependent Beta-Thalassemia Patients in Italy: A Retrospective Analysis

To evaluate demographic and clinical characteristics of transfusion-dependent beta-thalassemia (TDT) patients, the pattern of blood transfusion and iron-chelation therapy (ICT), healthcare-resource consumptions and healthcare direct costs in TDT patients. A retrospective study was carried out by integrating administrative databases of a pool of Italian Healthcare-Entities, with data reproportioned to the Italian population. Patients with ≥6 episodes of blood transfusion over a 12-month period together with ≥1 ICT prescription between 01/01/2010-31/12/2016, were included. Index-date was that of first blood transfusion within the inclusion period. Patients were characterized 12-month before index-date and followed-up from index-date to death or study-ending. Annual healthcare-resources/patient and direct healthcare-cost/patient [covered by NHS, for drugs, outpatients specialistic services (OSS), and hospitalizations] were evaluated during the follow-up. Overall, 2,137 TDT patients were estimated, with a mean age of 41.3 and 45% male. Previous hospitalizations were observed in 34% of patients. Anti-infectives drugs were the most prescribed (74%). During follow-up, hepatic diseases represented a complication in 38% of patients. Mean number of blood transfusions administrated over 12-month period was 11.9. Median time from first transfusion to first ICT prescription was 216 days. Concerning ICT treatments, 69% of patients were with the same ICT, while 26% and 5% presented 2-3 different types of ICT prescriptions, respectively. Mean annual number/patient of drug prescriptions was 23.4, of hospitalizations was 2.2 (median length of stay 82 days), and of OSS was 103.7. The overall annual direct healthcare-cost/patient averaged 20,343€ (9,832€ for drugs, 9,449€ for hospitalizations, and 1,062€ for OSS). This real-world study provided an in-depth characterization of the clinical features, pharmaco-utilization and economic burden of TDT patients, showing the high resource consumption and direct health-care costs for these patients. The mean age observed reflected the life-expectancy increment of TDT population reported in literature, suggesting that aging should be considered in the management of beta-thalassemia.

Blood transfusion therapy
 in cancer patients: modern aspects

Relevance: Anemia is a common syndrome in cancer patients, and blood transfusion is the most common
 treatment for anemia. Anemia and blood transfusion
 are associated with worse clinical outcomes. The efficacy and safety of different blood transfusion strategies
 are unclear.
 The purpose was to analyze current blood transfusion strategies in oncology.
 Results: In most clinical situations, a restrictive
 strategy showed efficacy and safety. However, low
 perioperative hemoglobin level is associated with increased mortality and complication rates. Several clinical trials demonstrated the benefits of liberal strategy;
 it may be due to comorbidities and low compensatory
 ability.
 Conclusion: In cancer patients, anemia is associated with worse clinical outcomes. Therefore, modern
 blood transfusion therapy strategies in oncology follow
 the restrictive approach, taking into account possible
 blood transfusion complications. Cancer patients are a
 complicated patient category and require an individual
 approach to postoperative anemia treatment.

Pattern of Blood Donation and Transfusion Transmissible Infections in a Hospital-Based Blood Transfusion Service in Lagos, Nigeria

Background: Blood transfusion therapy remains a mainstay in the care of many tropical diseases. Blood supply in sub-Saharan Africa (SSA) including Nigeria is still a challenge in terms of perennial blood shortages and transfusion transmissible infections (TTI). This study aimed to describe blood donation patterns and compare the rates of TTIs among types of blood donors seen at LUTH.
 Study method: A 6-year retrospective review of data on donor types and pattern of TTIs at LUTH transfusion service. TTI rates and odd ratios (OR) of TTI positivity amongst VNRD versus FRD were computed at 95% confidence intervals. Proportion of TTI sero-positivity was also compared between the VNRD and FRD groups.
 Results: A total of 53,319 blood donors were observed over the study period, with average of 8886.5 donors per year. VNRDs accounted for 12.3% (6533/53,319), while FRDs accounted for 87.7% (46,789/53,319) of all donors. The proportion of VNRDs ranged from 5.1% to 20.8%. The overall prevalence of TTIs over the 6-year period for all donors was 1.34% for HIV, 5.79% for HBV, 2.23% for HCV and 0.88% for syphilis. Sero-positivity rates for HIV, HBV, HCV, and syphilis was statistically significantly lower among VNRD compared to FRDs.
 Conclusion: There exists a lot of room for improvement in our blood donation practices to improve blood stock and transfusion safety. A more aggressive strategic effort needs to be directed towards achieving a target of 100% voluntary blood donor base through partnerships, advocacy, more financing and resource allocation, and other proven initiatives.
 Keywords: Nigeria; Transfusion; Transfusion transmitted infections; blood donors.

Seroprevalence of Viral Markers at the Blood Transfusion Center of Mohammed V Military Teaching Hospital of Rabat, Morocco

Introduction: Blood transfusion therapy has a huge role in management of various hematological and other diseases. The prevention of transfusion-transmitted infections remains a key component of blood safety. The main objective of this study is to study the seroprevalence of viral markers on donated blood at the Blood Transfusion Center, Mohammed V Military Hospital in Rabat. Material and methods: This is a retrospective study conducted with military donors aged 19 to 50 at the Blood Transfusion Center (CTS) of the Mohammed V Military Hospital of Instruction (HMIMV), Rabat (Morocco), carried out over a period of four years from January 1, 2017 to December 31, 2020. Biological screening was carried out by a technique…. based on the principle of Results: Out of 49,703 samples tested, 48,175 were valid for transfusion and 1,528 were not valid for transfusion, among these invalid bags, 101 donations were confirmed positive for HBV (2 ‰), with an HCV seroprevalence of 1.2 ‰ (58 donations) and a seroprevalence of 0.5 ‰ (25 cases) of HIV 1 + 2 with a seroprevalence of 0.5 ‰ (25 cases). Discussion: The low rates of seroprevalence of viral markers in our study show improved preventive measures with regard to donor selection and screening tests. Conclusion: This perceived prevalence encourages the maintenance of the use of the combined reagent, which is the only alternative to molecular biology for developing countries.

Incidence of Stroke and Other Brain Imaging Findings in Adult Patients with Sickle Cell Disease: A Retrospective Study

Background: Vaso-occlusion and hemolytic anemia are the main features of sickle cell disease. Central Nervous system complications of sickle cell disease (SCD) can include silent cerebral infarcts (SCI), hemorrhagic or ischemic strokes, seizures and Moyamoya syndrome. SCI is the most common neurological complications in SCD. SCI patients have abnormal Magnetic Resonance Imaging (MRI) of the brain with normal neurological examination and no history of stroke. More precisely, Casella et al in the Silent Cerebral Infarct Multi-Center Clinical trial (SIT) defined infarct as MRI signal abnormality that was at least 3 mm in one dimension and that was visible in two planes on fluid-attenuated inversion recovery T2 -weighted images, as determined by agreement of two of the three study neuroradiologists. SCI in children is associated with lower Intelligence Quotient and increased risk of stroke.Methods: In this retrospective study we reviewed 100 adult patients at our Comprehensive Sickle Cell Center. We looked at the incidence of documented stroke and other neurological complications in SCD patients. We reviewed the hospital records of SCD patients for any incidental brain imaging (MRI brain or Computed Tomography(CT) brain) findings, done as inpatient or in the emergency room as part of hospital visits.Results: Out of the 100 patients, 14 patients had documented stroke. On the review of other 86 patients, 10 patients had MRI brain, 23 patients had CT brain, and 53 patients had no brain imaging record. Out of the 10 patients with MRI brain, 7 patient had significant findings- 3 patients had small vessel ischemic changes, 1 patient with chronic petechial hemorrhage of cerebellar vermis, 1 patient with ischemic changes and Chiari malformation type 1 (CTM 1), 1 patient with CTM 1 and non-specific white matter changes and 1 patient with CTM 1 alone. Out of the 22 CT of the brain and 1 CT angiogram of brain, 2 imaging showed ischemic changes and the angiogram showed large cerebral vessel narrowing. Therefore, on our review, 19 patients out of 100 were found to have stroke and 24 out of 100 had significant neurological findings. However, only 14 out of 19 patients had documented stroke. 7 out the 10 patients with MRI brain had significant incidental neurological findings. The number of adult SCD patients with neurological findings in our cohort could have been higher, if we had brain imaging record for the 53 other patients.Conclusion: This study shows that there is a heavy burden of neurological complications in SCD patients and SCI is under-diagnosed. MRI brain is superior to CT brain in detecting neurological complications in SCD patients. SIT showed that regular blood-transfusion significantly reduced the recurrence of cerebral infarct in children with SCI. Finding of SCI in adults also warrants consideration for follow up brain imaging, neurology referral and discussion of treatment modalities including chronic blood transfusion and hydroxyurea therapy. CTM 1 can also be associated with SCD. In CTM 1, tonsil herniates 5 millimeter or below foramen magnum. SCD may cause increased thickness of calvarium from extramedullary hematopoiesis in skull, which can result in cranio-cephalic disproportion with cerebellar ectopia and subsequent CTM 1. Because of high incidence of neurological complications in SCD patients, routine screening with MRI brain should be considered in adult SCD patients. DisclosuresIdowu: Forma Therapeutics, Inc.: Research Funding; Pfizer: Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Ironwood: Research Funding.

Limited Access to Transcranial Doppler Screening and Stroke Prevention for Children with Sickle Cell Disease in Europe: Results of a Multinational Eurobloodnet Survey

Background:Children with sickle cell disease (SCD) are at increased risk of cerebrovascular events such as stroke, silent infarcts and neurocognitive impairment. The role of Transcranial Doppler ultrasound scanning (TCD) to identify sickle cell anemia (SCA) children at high risk of stroke is well established. Adam et al in 1998 recommended those with abnormal cerebrovascular flow velocities are offered prophylactic blood transfusion therapy to prevent stroke between ages 2 to 16 years. Therefore, TCD screening for stroke prevention in now is a mandatory in all guidelines for the management of children with SCA. However, there is still no uniform implementation of the program globally and in European countries (Rees 2016). Moreover, the information available on the quality of the TCD screening is limited to educational experiences in a few countries (Inusa 2019) but to evaluation of stroke prevention programs has been performed in Europe.As more disease modifying therapies become available for children with SCD, it is mandatory to know TCD availability, screening practices, and real-world data on stroke prevention in Europe.EuroBloodNet is the European Reference Network (ERN) on rare Hematological Disorders, one of the 24 ERN established by the European Union to improve care of patients with rare disorders in Europe. EuroBloodNet's main goal is to improve the healthcare and overall quality of life of patients with a Rare Hematological Disease by: 1) Improving equal access to highly specialized healthcare delivery through a multidisciplinary patient centered approach; 2) Enhancing the best practices in prevention, diagnosis and safe clinical care across Europe based on promotion of evidence based guidelines.We wanted to assess the state of the art of TCD screening and stroke prevention programs in European Expert Centers.Methods: An online survey was developed by SCD experts in 5 European countries and sent to all Representatives of the Health Care Providers (HCP) and the Red Cell Disorder representatives in each HCP within the EuroBloodNet network, as well as to National Representatives of Scientific Societies within European Countries. Items in the survey are listed in Table 1.Results81 hematologists or pediatricians from 77 centers in 16 European countries responded to the survey (14/16 in Western Europe); 39/77 (51%) were EuroBloodNet Expert centers, 14/77 (18%) were under evaluation as being recognized; 67/77 specified their expertise: 24% were pediatric, 3% adult, 58% both; 12 centers had >200 patients in the age range 1-16 years.36% Physicians reported not having a dedicated TCD/TCDi service for children with SCD so exams had to be performed by cardiologists (10%), general radiologists (28%), TCD is not performed (31%), or patients have to be sent in another center (31%). 74% reported requesting annual TCD for their patients, but to the question “What percentage of your patients receives annual TCD” only 28% confirmed that all their patients managed to actually receive annual TCD, due to lack of trained staff (43%), lack of TCD instruments (11%), refusal of patients due to logistical difficulties (22%) (i.e TCD in another city), lack of funds for dedicated staff or equipment (11%), or other reasons.Only 74% of hematologists were aware of the protocol in use at their center by the staff performing TCD; the STOP criteria were applied by 64% of the physicians, mainly due non evaluation of the Internal Carotid Artery. The extracranial part of the carotid artery was evaluated only in 30% of the respondents.In case of abnormal/conditional TCD results, the approach varies and is not uniform across centers.ConclusionsOur data show that less than 30% of children with SCD followed in European Centers receive annual TCD according to recognized guidelines. This first multinational European survey allowed the identification of issues related to the lack of access to TCD, lack of trained staff, lack of adequate protocols for implementation of TCD and treatment afterwards, which will need to be addressed through dedicated actions. [Display omitted] DisclosuresCasale: Novartis Farma SpA: Honoraria, Membership on an entity's Board of Directors or advisory committees. Mañú Pereira: Novartis: Research Funding; Agios Pharmaceuticals: Research Funding. de Montalembert: Vertex: Membership on an entity's Board of Directors or advisory committees; BlueBirdBio: Membership on an entity's Board of Directors or advisory committees; Addmedica: Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees. Colombatti: Global Blood Therapeutics: Research Funding; Addmedica: Consultancy; Forma Therapeutics: Consultancy; Novartis: Consultancy; NovoNordisk: Consultancy; BlueBirdBio: Consultancy; Global Blood Therapeutics: Consultancy; BlueBirdBio: Research Funding.

Pengaruh Lama Penyimpanan Kantong Darah terhadap Kadar Hemoglobin pada Komponen Whole Blood di Unit Donor Darah PMI Kota Palangka Raya

Hemoglobin levels are used as a requisite for determining blood transfusion therapy for patients with anemia. Prior to blood transfusion, it is initially stored in blood bags that contain CPDA-1 anticoagulant with a shelf life for 35 days at temperature 2-6°C with a FIFO system. The purpose of this study was to determine hemoglobin levels in blood bags on the third dan fifth day of increasing the patient’s hemoglobin level in Unit Donor Darah PMI Palangkaraya. The research was conducted with a descriptive method with a Linear Regression research design. The sample used is a whole blood bag of blood obtained by purposive sampling technique with a total sample is 30 blood bags. The paired Linear Regression on the while the whole blood bags obtained p < 0.05 indicating that there was effet storage in hemoglobin levels on the third day, and p > 0.05 indicating that was no effect storage in hemoglobin levels on the fifth day.

Interrelationship between liver T2*-weighted magnetic resonance imaging and acoustic radiation force impulse elastography measurement results and plasma ferritin levels in children with β-thalassemia major.

To evaluate correlation and agreement between T2*-weighted magnetic resonance imaging (T2*-wMRI), acoustic radiation force impulse elastography (ARFI-e) measurement results of liver and plasma ferritin levels (PFLs) in children with β-thalassemia major (β-TM). The study included 40 pediatric patients (aged 64-216 months; 14 girls, 26 boys) receiving blood transfusion and chelation therapy. To detect the severity of liver iron overload (LIO) and concomitant parenchymal fibrosis, T2*-wMRI and ARFI-e measurements were performed from the right lobe segments. Student's t-test, Mann-Whitney U, ANOVA, Spearman's test and ICC were used for statistical analysis. After the measurements of T2*-wMRI, patients were grouped as normal in 4 (10%), mild in 11 (27.5%), moderate in 21 (52.5%), and severe in 4 (10%) cases in terms of LIO. Combined moderate and severe groups had significantly higher ARFI-e and PFL values than the combination of other groups (p=.001, p=.040). The ARFI-e measurements of boys were found to be significantly higher than those of girls (p=.023). A strong negative correlation between T2*-wMRI and ARFI-e and a moderate negative correlation between T2*-wMRI and PFL were detected (p;r=0.001;-0.606, p;r=0.009; -0.407). A strong positive correlation was found between ARFI-e values and PFL (p;r=0.001; 0.659). The optimal cut-off value of ARFI-e to predict liver fibrosis because of moderate&severe LIO was determined to be 1.29 M/s (80% sensitivity and 88% specificity). A moderate agreement was observed between the T2*-wMRI and ARFI-e methods [ICC: 0.680, 95% CI: (0.470 to 0.817)]. Given the strong correlation and moderate agreement between ARFI-e and T2*-wMRI, ARFI -e could be used to monitor LIO in children with β-TM.

Результати дослідження морфологічних особливостей і серотоніндепонувальної функції тромбоцитів у донорів крові

Blood transfusion service and its social component – donorship must be the priority areas of the state policy because the results of its work are of paramount importance. The main task of the blood transfusion service is supply of high quality components for blood transfusion therapy. Quality of blood components is compliance of properties and specifications of the blood component supplied to the recipient with the set standards. Strict order of conformance with the approved regulations and procedures is important at all technological states and is a cornerstone of blood transfusion service products quality. All actions, planned and implemented, starting with planning donorship and ending with the finished product manufacturing and storage conditions, are important for ensuring the quality as the final result. The purpose of the study was to determine the content of total, platelet and free serotonin in the plasma of primary and active blood donors for further use of the studied parameters to assess the quality of platelet concentrate. Materials and methods. 160 blood donors (118 men and 42 women) were examined, including 110 active donors (85 men and 25 women) who donated blood regularly at least 3 times a year and 50 primary reserve donors (32 men and 18 women), who donated blood for the first time. Primary reserve donors formed a control group. For the convenience of systematization and reproduction of scientific research results, objectification in comparing research data, all examined active donors, depending on the duration of donor experience and, accordingly, increasing the probability of occurrence of hidden platelet metabolism disorder, were divided into three subgroups: subgroup I – 51 donors (39 men and 12 women), whose donor experience lasted from 2 to 5 years; subgroup II – 31 donors (24 men and 7 women), duration of donor experience which ranged from 6 to 9 years; subgroup III – 28 donors (22 men and 6 women), whose donor experience lasted 10 years or more. The method of fractional determination of biological amines – serotonin – is presented in preliminarily dried biostrate specimens. The method described includes some extraction procedures with optimal controlled pH values necessary for isolating serotonin, producing fluorophors in accordance with orthophtaldialdehide and ninhydrin, the subsequent fluorimetric estimation of their levels being performed on the native fluorometer «БИАН». Results and discussion. It was found that active donors compared to control, have a level of free serotonin in peripheral blood platelets which was significantly higher. Biochemical shifts were revealed in the background of certain morphological changes of platelets. Possible pathophysiological mechanisms of the detected changes are discussed in the article. Conclusion. Active blood donation is accompanied by significant changes in the morphological parameters of platelets and the content of free serotonin in peripheral blood platelets. Further study of platelet hematopoiesis of active blood donors is required

The clinical profile and outcome of children with transfusion-dependent thalassaemia in KwaZulu-Natal Province, South Africa: A 16-year retrospective review

Background . The clinical presentation and the outcome of patients with transfusion-dependent thalassaemia (TDT) in the province of KwaZulu-Natal, South Africa, have not been previously reported. Objectives. To document the clinical characteristics, treatment strategies, outcomes and complications from a case series of children with TDT. Method. A retrospective chart review of the clinical hospital records, laboratory, and blood transfusion data of patients with beta thalassaemia was performed. Data on serum ferritin (SF) levels, magnetic resonance imaging (MRI) R2 and/or T2* radiological tests to assess the presence of iron overload were collated and analysed. Iron chelator medications and haematopoietic stem cell transplantation (HSCT) outcomes were reported. Results. All the 12 children enrolled with TDT were of Indian descent. All patients had regular blood transfusion and iron chelation therapy (ICT). Four patients had iron overload diagnosed on SF, liver and cardiac MRI scanning. Most of the children in this study were either initiated or changed to oral deferasirox (DFX) during treatment follow-up. One child on parenteral iron chelation deferoxamine, with high SF levels, died. Four patients successfully underwent HSCT without complications and are in remission. Conclusion. The study confirms that haemoglobin-level-driven blood transfusions with adequate chelation are possible in the public health service in South Africa. Close monitoring for iron overload is required, using a combination of SF and MRI scanning. Oral ICTs are the preferred agents for reducing iron overload. HSCT remains the treatment of choice for TDT when feasible.

Economic evaluation of regular transfusions for cerebral infarct recurrence in the Silent Cerebral Infarct Transfusion Trial

AbstractIn 2020, the American Society of Hematology published evidence-based guidelines for cerebrovascular disease in individuals with sickle cell anemia (SCA). Although the guidelines were based on National Institutes of Health–sponsored randomized controlled trials, no cost-effectiveness analysis was completed for children with SCA and silent cerebral infarcts. We conducted a cost-effectiveness analysis comparing regular blood transfusion vs standard care using SIT (Silent Cerebral Infarct Transfusion) Trial participants. This analysis included a modified societal perspective with direct costs (hospitalization, emergency department visit, transfusion, outpatient care, and iron chelation) and indirect costs (special education). Direct medical costs were estimated from hospitalizations from SIT hospitals and unlinked aggregated hospital and outpatient costs from SIT sites by using the Pediatric Health Information System. Indirect costs were estimated from published literature. Effectiveness was prevention of infarct recurrence. An incremental cost-effectiveness ratio using a 3-year time horizon (mean SIT Trial participant follow-up) compared transfusion vs standard care. A total of 196 participants received transfusions (n = 90) or standard care (n = 106), with a mean age of 10.0 years. Annual hospitalization costs were reduced by 54% for transfusions vs standard care ($4929 vs $10 802), but transfusion group outpatient costs added $22 454 to $137 022 per year. Special education cost savings were $2634 over 3 years for every infarct prevented. Transfusion therapy had an incremental cost-effectiveness ratio of $22 025 per infarct prevented. Children with preexisting silent cerebral infarcts receiving blood transfusions had lower hospitalization costs but higher outpatient costs, primarily associated with the oral iron chelator deferasirox. Regular blood transfusion therapy is cost-effective for infarct recurrence in children with SCA. This trial is registered at www.clinicaltrials.gov as #NCT00072761.

A study on quality of life among thalassemic children aged 8 to 18 years

Background: The assessment of quality of life in children, especially in those with chronic illness such as thalassaemia, is particularly important. It differs from other forms of medical assessment in that it focuses on the individuals’ own views of their well-being and other aspects of life, giving a more holistic view of well-being.Methods: Hospital based descriptive, observational study was conducted on 200 school-age children. Two tools were used to collect the necessary data. The first was a structured interview questionnaire sheet including socio-demographic data of children and their parents as well as medical history. The second tool was a standardized tool (Pediatric Quality of Life Inventory TM Version 4.0).Results: The results of the present study revealed that the quality of life of school-age children with Thalassemia Major was affected. According to child’s report regarding total QOL score 30% of children had good score compared to 35% in parent’s report, while 65% had fair score compared to 60% in parent’s report. Emotional functioning scored the lowest followed by physical then school and social functioning. Regarding compliance with treatment, 91% of the studied children had regular blood transfusion therapy. In addition, 81% of the studied children were compliant with iron chelation therapy.Conclusions: Thalassemia has a negative impact on perceived physical, emotional, social and school functioning in thalassemia patients. There was a significant association between the total quality of life and compliance with blood transfusion and regular iron chelation therapy in both child and parent report.

Low‐cost alternatives for the management of Guillain‐Barré syndrome in low‐ and middle‐income countries

AbstractGuillain‐Barré syndrome (GBS) is the most common cause of acute flaccid paralysis. It can cause rapidly progressing ascending paralysis leading to respiratory failure. There is no definite treatment for GBS, but early administration of intravenous immunoglobulin or therapeutic plasma exchange accelerates motor recovery and decreases time on the ventilator support and are considered the standard of care in GBS. However, these treatments are often unaffordable for many patients in low‐ and middle‐income countries, with limited availability and specialized expertise required in prescription. Cheaper alternatives such as modified therapeutic plasma exchange, exchange blood transfusion, rituximab, and pulse steroid therapy have been practiced in different parts of the world and have been shown to be effective, albeit with limited evidence. These cost‐effective therapies warrant further research and should be strongly considered by relevant health authorities and policymakers as a potential primary treatment for GBS in resource‐limited settings.

Impact of Allogeneic Leukocyte-Depleted Red Blood Cell Transfusion on Inflammatory Response and Blood Coagulation in Patients with Recurrence of Colon Cancer after Operation.

Objective Anemia inevitably affects the survival of cancer patients. In clinical practice, patients with anemia and decreased blood volume are treated by component blood transfusion. Through targeted blood transfusion therapy, the efficacy of blood transfusion treatment can be validly improved, which renders clinical benefits in reducing transfusion-induced adverse reactions (ARs). This research project mainly investigated the impact of allogeneic leukocyte-depleted red blood cell (LDRBC) transfusion on inflammatory response and coagulation status of patients with postoperative recurrence of colon cancer (CC). Methods A total of 80 patients with postoperative recurrence of CC admitted to Changzhou Second People's Hospital affiliated to Nanjing Medical University from September 2017 to December 2020 were selected as the study subjects. 36 patients with allogeneic suspended red blood cell (RBC) transfusion were used as the control group (CG), and 44 patients receiving allogeneic LDRBC transfusion served as the observation group (OG). The two groups were compared regarding alterations in pretransfusion and posttransfusion serum inflammatory factors, stress indicators and coagulation function, incidence of ARs, postoperative infection, and average incision healing time. Results After blood transfusion, serum tumor necrosis interleukin- (IL-) 6, IL-10, factor-α (TNF-α), and C-reactive protein (CRP) decreased in both cohorts, with lower parameters in CG (P < 0.05); the stress indexes, adrenocortical hormone (ACTH), adrenaline (AD), norepinephrine (NE), and cortisol (Cor) increased, especially in CG (P < 0.05); prothrombin time (PT), thrombin time (TT), and activated partial prothrombin time (APTT) in CG were lower than those in OG, and FIB was higher than that in OG with significant differences between two cohorts (all P < 0.05). The two groups had similar cases of lung infection and wound infection (P > 0.05), but the incision healing time was evidently shorter in OG as compared to CG (9.73 ± 1.86 vs 14.67 ± 2.39 d, P < 0.05). The total incidence of ARs was 16.7% in CG and 6.9% in OG, with no significant difference (P > 0.05). Conclusions In the blood transfusion treatment for patients with postoperative recurrence of CC, LDRBC transfusion renders significant clinical benefits, which can effectively improve the coagulation function of patients, reduce stress reaction, and shorten incision healing time without increasing ARs during treatment and postoperative infection rate, which is worth popularizing.