Transfusion Therapy In Patients Research Articles

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

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

5613354 SCREENING FOR CEREBROVASCULAR DISEASE IN CHILDREN WITH SICKLE CELL DISEASE USING TRANSCRANIAL DOPPLER AND MRA TO PREVENT STROKE: THE AMSTERDAM COHORT

Background: Cerebrovascular complications are the most devastating complications in patients with Sickle Cell Disease (SCD). To identify patients at risk of a first cerebrovascular accident (CVA), the American Society of Hematology (ASH) 2020 guideline for SCD recommends annual transcranial Doppler ultrasonography (TCD) screening for children aged 2-16 years with HbSS or HbSβ0 thalassemia. When abnormal TCD velocities are detected, chronic transfusion therapy is advised for at least a year.(1) Since 2000 we have implemented a different management protocol in Amsterdam that only prescribes transfusion therapy for children with confirmed MRA abnormalities when increased flow is present on TCD measurement. This Amsterdam protocol reduces exposure to chronic blood transfusions but we need to investigate the safety and efficacy. Methods: In this longitudinal single center cohort study, all patients managed according to the Amsterdam Protocol between January 2000 and September 2022 were included. TCD screening was performed in children with genotypes SS or Sβ0-thalassemia at a frequency of twice a year in the age group 4-10 years and annually in children aged 10 to 18 years. From January 1st 2010 onwards, screening was already initiated at the age of 2 years. When TCD screening demonstrated abnormal results at 2 separate assessments, magnetic resonance angiography (MRA) was performed. We only started chronic transfusion therapy in patients with a stenotic lesion present on MRA. In patients without abnormalities on MRA follow-up by TCD according to protocol was continued. Results: A total of 209 SCD patients were included in this study, cumulative 2874.5 patient-years of follow-up. The median age of these patients at the start of the follow-up was 0.74 (IQR: 0.0, 5.5) years. In this cohort, 30 patients had abnormal TCD results at 2 separate assessments. In all of these patients MRA was performed, which showed abnormal results in 16 cases. None of the 12 patients with abnormal TCD results and normal MRA results who did not receive chronic transfusion therapy developed a CVA. The total follow-up duration after the first abnormal TCD in these patients was 112.0 patient-years. Nine out of the 14 patients (64%) who had abnormal TCD results used hydroxyurea. The median time between the first abnormal TCD and initiation of hydroxyurea therapy was 3.9 (IQR: 3.0, 5.2) years. Two patients with abnormal TCD and normal MRA results were treated with chronic transfusion therapy. In these cases, chronic transfusion therapy was started because of recurrent acute chest syndrome (ACS) and abnormal TCD results (despite normal MRA results) respectively. Discussion: These data suggest that treatment according to the Amsterdam Protocol is a safe and efficient way to prevent primary stroke in children with SCD and to reduce exposure of these patients to long term transfusion therapy. Although the group of patients in this cohort with abnormal TCD velocities and normal MRA that did not receive transfusion therapy is limited to 12 patients with cumulative 112.0 patient-years of follow-up, we believe the results of this study are reassuring, since the cumulative incidence rate in this study is significantly lower (0.0 (95% CI: 0.0, 0.25)) compared to the incidence reported for patients with abnormal Doppler results in the original study by Adams et al. (0.4 (95% CI: 0.27, 0.59) at 30 months).(2)

Sec14-like-Lipid-Binding-4 Enables Hemoglobin Clearance By Liver Sinusoidal Endothelial Cells

Hemolysis of banked red blood cells (RBC storage lesion) contributes to lack of therapeutic benefit and higher mortality following transfusion therapy in patients. However, the molecular mechanism underlying storage lesion remains poorly understood. Using a multiple-ancestry genome-wide association analysis among blood donors, our group has recently identified Sec14-like-lipid-binding-4 (Sec14L4) protein to be associated with increased rate of hemolysis following blood storage. Although Sec14L4 is highly similar to yeast phosphatidylinositol transfer protein Sec14, which is known to be involved in the synthesis of golgi-derived transport vesicles, the exact function of Sec14L4 and how it promotes RBC storage lesions remains unknown. Our initial studies using fluorescence microscopy in our newly generated Sec14L4 null mice revealed significant enrichment of Sec14L4 in the live sinusoidal endothelial cells (LSECs), which positively correlated with elevated levels of iron in the liver. Scanning and transmission electron micrographs revealed impaired actin cytoskeleton organization in LSECs of Sec14L4 null mice, which was associated with upregulation of major GTPases RhoA, RhoB and Rac1 activity. The impairment of actin cytoskeletal organization in Sec14L4 null mice lead to impaired vesicle trafficking in LSECs and higher retention of RBCs in the liver sinusoids compared to control mice. These data are the first to demonstrate a previously unrecognized role for Sec14L4 protein in LSEC mediated endocytic trafficking by promoting actin polymerization. These findings also suggest that Sec14L4 may regulate RBC storage lesion through impaired hemoglobin clearance by LSECs secondary to cytoskeletal impairment.

Comparative retrospective study on the validity of point-of-care testing device for massive obstetrical hemorrhage: dry hematology vs thromboelastography

Early recognition of hypofibrinogenemia and prompt initiation of transfusion therapy in patients with massive obstetrical hemorrhage can improve prognosis. There are reports on the usefulness of point-of-care testing, which provides quicker test results compared with fibrinogen measurements using the conventional Clauss method. This study aimed to compare and investigate the diagnostic accuracy of dry hematology and thromboelastography in point-of-care testing for the diagnosis of hypofibrinogenemia. A single-center, retrospective study of 126 massive obstetrical hemorrhage cases with point-of-care testing before treatment was initiated. The correlation of fibrinogen values with the Clauss method and the diagnostic accuracy for hypofibrinogenemia were compared between dry hematology and thromboelastography. Fibrinogen value in dry hematology showed a strong positive correlation with values measured by the Clauss method, and the diagnostic accuracy for hypofibrinogenemia was high, but there were many residuals above 100 mg/dL, and the distribution of these residuals was not uniform. Although thromboelastography cannot be used to directly measure fibrinogen values, maximum amplitude citrated functional fibrinogen, amplitude-10 citrated rapid thromboelastography, and amplitude-10 citrated functional fibrinogen showed a strong positive correlation with fibrinogen values using the Clauss method, and no significant difference in correlation or diagnostic accuracy was observed relative to dry hematology. Dry hematology and thromboelastography were equally accurate in diagnosing hypofibrinogenemia, with results correlating well with fibrinogen values measured by the Clauss method.

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.

Improving Completion Rates of Transcranial Doppler Ultrasounds in Children with Sickle Cell Disease Using Quality Improvement Efforts: In-Clinic Vs. Population-Based Assessments

Introduction:People living with sickle cell disease (SCD) are at risk for stroke due to progressive cerebral vasculopathy. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) study showed that transfusion therapy in patients with abnormal cerebral blood flow velocities measured by transcranial doppler (TCD) ultrasound significantly reduced the risk for stroke. Based on the STOP and STOP II studies, the National Heart, Lung and Blood Institute (NHLBI) and American Society of Hematology (ASH) recommend annual TCD screenings for children with HbSS and HbSb0 genotypes from age 2-16. Despite this recommendation, studies show that fewer than half of eligible children with SCD complete annual TCD screening. Recently, Cabana and Treadwell et al. (2020) found high TCD referral and completion rates in a multi-site quality improvement (QI) initiative (85% baseline) using chart review. Another feasible approach to tracking guideline adherence uses administrative claims data which are derived from diagnostic and billing codes from statewide claims. Claims data can be used to assess population estimates for a clinic or a state, for all eligible patients, including those who may not routinely access care. In this study, we use administrative claims to assess TCD completion rates in the same clinics participating in the aforementioned QI initiative. We hypothesized that, population level rates would be lower than those assessed via chart review and that QI strategies may not lead to sustained TCD completion rates.Methods:Between August 2017 to August 2018, a QI initiative within the Pacific Sickle Cell Regional Collaborative (PSCRC) was conducted to improve referral and completion rates of TCD screenings. Site leads participated in a monthly QI learning collaborative, implementing and reporting on Plan-Do-Study-Act (PDSA) cycles, with bimonthly chart review data collection. Medicaid administrative claims from the four states with participating clinics, from 2017, 2018 (to assess baseline and post-QI initiative performance) and 2019 (to assess sustainability), were used to assess rates of TCD completion in the eligible pediatric population, using the specifications of a previously validated quality measure by Reeves et al (2019). Annual TCD completion rates and changes in completion rates over time were assessed for each site and state.Results:Five sites from four states in the PSCRC were included in the analysis. There was large variability in the number of eligible patients in each clinic (13-75) and state (23-588). Based on administrative claims, TCD clinic-level completion rates at baseline ranged from 41.7% to 69.2% at individual clinics. After 12 months of QI participation, TCD completion rates improved at allsites (range 4.6% to 29.2%). The site with the largest change improved TCD completion rate from 41.7% to 70.8% (n=24). All but one site had a decrease in TCD completion rate after completing the QI initiative and in 2019, TCD completion rates were within 10% of baseline completion rates at all sites (range -8.2% to 8.3%). At the statewide level, one state had a sustained improvement in TCD completion (improvement from baseline: 8.8%). In three of the four states providing data, TCD completion rates decreased from baseline (range -0.7% to -12.6%).Discussion:In a regional collaborative, we found improvements in TCD completion in the setting of a QI initiative focused on TCD, which were not sustained in the year after. This suggests the need for a systems-level approach to improvement, leading to feasible sustainability when no longer the focus of a collaborative. In addition, our data show that, when using administrative claims, rates of TCD completion are lower than rates when using chart review data (41.7% to 69.2% vs. 85% by chart review noted in prior publication). Thus, while site-specific medical record review provides insight into the quality of care for patients seen in the clinic, administrative claims data allow for a global understanding of the quality of care for the clinic population at risk, including those who do not attend clinic regularly. This suggests additional potential focus for quality improvement initiatives, such as systems to optimize outreach to patients who may not routinely access care. This type of outreach may best be done by health plans, potentially in partnership with sickle cell specialists, and would be an important tool for improving health for children with SCD. DisclosuresVissa: Global Blood Therapeutics Inc: Research Funding. Treadwell: National Alliance of Sickle Cell Centers: Other: Early Evaluation of the Use of Crizanlizumab in Sickle Cell Disease.

Effect of cryoprecipitate transfusion therapy in patients with postpartum hemorrhage: a retrospective cohort study

To evaluate the effect of cryoprecipitate (CRYO) transfusion in women referred for postpartum hemorrhage (PPH). This retrospective cohort study included patients with primary PPH referred to Gifu University Hospital between April 2013 and March 2020. We analyzed the effect of CRYO transfusion on fluid balance 24 h after the initial examination using a multivariable linear regression model adjusted for several confounding variables. To evaluate whether outcomes were modified by active bleeding, an interaction term of CRYO*active bleeding was incorporated into the multivariable model. We identified 157 women: 38 in the CRYO group (cases) and 119 in the control group. Fluid balance in the aforementioned period tended to decrease in the CRYO group compared with that in the control group (coefficient − 398.91; 95% CI − 1298.08 to + 500.26; p = 0.382). Active bleeding on contrast-enhanced computed tomography affected the relationship between CRYO transfusion and fluid balance (p = 0.016). Other outcomes, except for the overall transfusion requirement, were not significantly different; however, the interaction effect of active bleeding was significant (p = 0.016). CRYO transfusion may decrease the fluid balance in the first 24 h in PPH patients, especially in those without active bleeding.

1443MO Differences in hematologists’ and palliative care physicians’ recommended indications and opinions on transfusion therapy for patients with hematological malignancy post-anticancer therapy

Transfusion therapy occasionally impedes the transition of patients with hematological malignancies to specialized palliative care. This study investigated differences in the opinions of hematologists (HOs) and palliative care physicians (PCs) regarding indications for end-of-life transfusion therapy.

P0329 / #717: COMPARISON OF TRANSFUSIONAL PRACTICE WITH VISCOELASTIC TESTS VERSUS CONVENTIONAL COAGULATION TESTS IN CONGENITAL HEART SURGERY PATIENT

Aims & Objectives: Analyze transfusion therapy in patients with congenital heart disease who undergo cardiovascular surgery and admitted to Pediatric Cardiac Intensive Care Unit (PCICU) UMAE CMN Siglo XXI Cardiology Hospital during the study period. Methods: Patients over 4 years of age up to adult age with congenital heart disease scheduled for elective cardiac surgery at the UMAE Cardiology Hospital of the National Medical Center “Siglo XXI” of the Mexican Social Security Institute (IMSS) in Mexico City, during the study period (May - December 2019). Cohort, observational, comparative, prospective, longitudinal study. Results: There were 98 patients, female sex prevailed with 57%, median age of 21 years. Predominating acianogenic heart disease (57%) such as interatrial communication. 26% of the patients were transfused, 8% had viscoelastic tests, the rest had conventional coagulation tests, 8 patients had significant post-surgical bleeding, performing viscoelastic tests using a management algorithm, only 2 were the patients who required surgical reintervention. Conclusions: In our unit, blood components continue to be transfused based on conventional coagulation tests. Viscoelastic tests applied to patients who presented significant postsurgical bleeding in our study significantly reduced surgical reoperation for bleeding. The viscoelastic test management algorithm is applied in 100% of cases.

Costs associated with transfusion therapy in patients with myelodysplastic syndromes in Sweden: a nationwide retrospective cohort study.

Background and objectivesBlood transfusion is a cornerstone therapy for many patients with myelodysplastic syndromes (MDS), but ranges from few to no transfusions to intensive transfusion therapy. To date, no large studies have described transfusion use or costs for patients with MDS, accounting for the range of disease severity.Materials and methodsA nationwide cohort study was conducted with all patients diagnosed with MDS in Sweden between 2008 and 2017, based on the Swedish MDS register and the Swedish part of the Scandinavian Donations and Transfusions Database 3 (SCANDAT3‐S). Patients were followed from diagnosis until death, emigration, allogeneic hematopoietic stem cell transplantation or end of follow‐up. Average cumulative transfusion count and costs over time were calculated, stratified by the revised international prognostic scoring system (IPSS‐R) and age at diagnosis. Costs calculations used data on incident transfusions and laboratory testing and were divided into: direct material costs, direct labour costs and laboratory costs.ResultsIn total, 2311 patients were included in the cohort. In the first four years after diagnosis, patients in the very low IPSS‐R category received on average 25 red cell (95% confidence interval, 20–32) and 4 (3–7) platelet transfusions. Conversely, patients in the very high‐risk category received on average 171 (135–200) red cell and 66 (51–78) platelet transfusions. Correspondingly, transfusion costs ranged from $8805 ($6482–$11 625) to $80 106 ($61 460–$95 792).ConclusionTransfusion count and costs for patients with MDS increased markedly with IPSS‐R risk category, but were similar across age groups. Transfusion costs were considerable for the highest IPSS‐R risk categories.

Platelet transfusion for patients with platelet dysfunction: effectiveness, mechanisms, and unanswered questions.

In this review, we discuss current clinical guidelines and potential underlying mechanisms regarding platelet transfusion therapy in patients at risk of bleeding, comparing management of patients with thrombocytopenia versus those with qualitative platelet disorders. Platelet transfusion therapy is highly effective in managing bleeding in patients with hypoproliferative thrombocytopenia. Clinical trials have demonstrated that platelet transfusion can be used at a lower trigger threshold and reduced platelet doses, and may be used therapeutically rather than prophylactically in some situations, although additional data are needed. In patients with inherited platelet disorders such as Glanzmann's Thrombasthenia or those with RASGRP2 mutations, platelet transfusion may be ineffective because of competition between transfused and endogenous platelets at the site of vascular injury. Successful management of these patients may require transfusion of additional platelet units, or mechanism-driven combination therapy with other pro-hemostatic agents. In patients on antiplatelet therapy, timing of transfusion and inhibitor mechanism-of-action are key in determining therapeutic success. Expanding our understanding of the mechanisms by which transfused platelets exert their pro-hemostatic function in various bleeding disorders will improve the appropriate use of platelet transfusion.

Clinical significance of nucleated red blood cell count in pediatric patients with transfusion-dependent beta thalassemia

Objectives The aim of this study was to assess the count of nucleated red blood cells (NRBCs) as a potential marker of erythropoietic stress and transfusion therapy adequacy. To achieve this goal, we evaluated the performance of the Sysmex XN-1000 hematology analyzer and flow cytometry (FC) in counting NRBCs in comparison with manual microscopic counting (MC). Background NRBCs recognition by manual and automatic technologies has been widely evaluated. As transfusion of packed red blood cells is a principal supportive intervention for patients with thalassemia major, monitoring markers of an adequate transfusion therapy is essential in their management. Patients and methods NRBC percentages from 61 thalassemia major patients were analyzed by automated technologies, Sysmex XN-1000 analyzer and FC, and the results were compared with traditional MC as a reference method. Markers of ineffective erythropoiesis were estimated for all patients. Results Pretransfusion hemoglobin levels showed significant negative correlation with NRBC% obtained by Sysmex XN-1000 (r=−0.302, P = 0.018). Furthermore, NRBC% was positively correlated with reticulocyte% (r = 0.852, P Conclusion Monitoring of NRBC% by Sysmex XN-1000 analyzer has many advantages over manual counting and can help to optimize transfusion therapy for patients with beta thalassemia major.

Performance characteristics of thromboelastometry assays using incompletely filled and prolonged stored samples.

Thromboelastometry (TEM) is often used to guide transfusion therapy in patients with massive bleeding. The effect of testing incompletely filled samples and those stored for a prolonged time at 4°C was investigated. Whole blood samples were collected from 15 healthy blood donors and were pooled according to ABO group. From these pools, aliquots were taken and diluted to produce final whole blood:citrate buffer ratios ranging from 90:10 (fully filled sample) to 40:60 (extremely under filled samples). These samples were then tested by EXTEM, INTEM, and FIBTEM on calibrated ROTEM delta machines. Separately, the four samples at 90:10 dilution were kept at 4°C for 16-20 hours and then retested on the ROTEM machines. All of the samples at the 90:10 and 80:20 (half-filled sample) whole blood:citrate buffer dilutions demonstrated ROTEM parameters within their respective reference ranges, although the samples from the 80:20 dilution tended to demonstrate slightly longer or slower times, depending on each ROTEM parameter, compared to the completely filled samples. All of the samples with more dilute whole blood to citrate buffer ratios (i.e., 70:30 to 40:60) yielded abnormal TEM results. The TEM results for the 90:10 dilution samples exposed to 16-20 hours of storage at 4°C were within the reference intervals. Completely and half-filled samples, and completely filled samples after prolonged cold storage, produced normal ROTEM results. Tubes that are less than half-filled should not be used for ROTEM testing.

Sonoclot Analyzer Guided Transfusion Therapy in Patients Undergoing On-Pump Cardiac Surgeries

Sonoclot Analyzer Guided Transfusion Therapy in Patients Undergoing On-Pump Cardiac Surgeries

Clinical efficacy of platelet transfusion therapy in patients with leukemia and analysis of risk factors for ineffective transfusion.

Clinical efficacy of platelet transfusion therapy in patients with leukemia was investigated to analyze risk factors for ineffective transfusion. A total of 105 cases of patients with leukemia admitted to The First Affiliated Hospital of Bengbu Medical College from August 2016 to November 2018 were selected as research subjects. A total of 49 patients received transfusion of apheresis platelet suspension, and were group A. Fifty-six patients who received mixed multi-person platelet suspension were group B. The percentage plate recovery (PPR) and corrected count increment (CCI), interleukin-11 (IL-11) and soluble glycoprotein 130 (sgp130) levels were compared between the two groups, and the correlation of PPR and CCI with serum IL-11 and sgp130 levels was analyzed. Multivariate logistic regression was used to analyze the independent risk factors affecting ineffective transfusion in patients with leukemia. After transfusion, PPR and CCI in both groups were significantly higher than those before transfusion (P<0.05). IL-11 was significantly increased in both groups after transfusion, and sgp130 was significantly decreased in the two groups after transfusion (P<0.05). Serum IL-11 level in leukemia patients was positively correlated with PPR and CCI (r=0.7693, P<0.001), (r=0.7760, P<0.001), and serum sgp130 level in leukemia patients was negatively correlated with PPR and CCI (r=-0.7086, P<0.001), (r=-0.7733, P<0.001). There were differences between the effective group and the ineffective group in transfusion frequency, fever, infection and splenomegaly (P<0.05). Fever (OR, 0.382; 95% CI, 0.183-0.972) and infection (OR, 0.367; 95% CI, 0.140-0.956) were independent risk factors for ineffective transfusion. In conclusion, apheresis platelet or mixed multi-person platelet suspension transfusion can significantly improve the disorder of platelet count in patients with leukemia, and improve the clinical efficacy. Fever and infection are independent risk factors leading to ineffective transfusion.

Plasma BDNF Levels Are Associated with Stroke in Children with SCD

Background: Cerebrovascular disease, particularly overt stroke, is one of the most critical clinical complications of sickle cell disease (SCD). Individual stroke risk for patients with SCD is assessed by measuring trans-cranial Doppler ultrasound (TCD) velocity. While TCD screening and resulting use of chronic blood transfusion therapy in patients with abnormal TCDs has reduced stroke incidence in patients with SCD from 11% to 1%, the test has significant limitations. It can only be performed on children old enough to remain still for the assessment, or young enough to have open bony windows; typically 2-16 years of age. Additionally the test has poor positive predictive value, causing patients to be placed on chronic transfusion therapy who would not have gone on to have a stroke. Hydroxyurea (HU) is replacing chronic transfusion therapy in many institutions for primary stroke prevention, but many sites initiate a "cooling off" period of transfusions prior to initiating HU, and transfusion therapy may be indicated for abnormal TCD velocities in patients already on HU. The pathophysiology of stroke in SCD patients is not completely understood, but vascular remodeling, abnormal cerebral blood flow, and vaso-occlusion all play a role. Plasma levels of Brain derived neurotrophic factor (BDNF), myeloperoxidase (MPO), vascular cell adhesion molecule 1 (VCAM-1) and intercellular adhesion molecule 1 (ICAM-1) have been shown to be associated with high trans-cranial Doppler ultrasound (TCD) velocity in children with SCD; but there is limited data on their potential association with stroke in this population. BDNF is a neurotropin that is responsive to hypoxia and promotes neurogenesis as well as playing a key role in the regulation of the cell survival pathway; myeloperoxidase (MPO) promotes vascular injury by oxygen radical generation, leukocyte/neutrophil recruitment, and oxidative stress; vascular cell adhesion molecule 1 (VCAM-1) is and endothelial adhesion molecule involved in the pathophysiology of vaso-occlusion in SCD through promoting RBC adhesion; and intercellular adhesion molecule 1 (ICAM-1) is expressed on endothelial and immune system cells and is thought to be a ligand for leukocyte adhesion. We hypothesized that BDNF, MPO, VCAM-1 and ICAM-1 may be involved in stroke in SCD, and may be potential biomarkers to be used to assess an individual with SCD's stroke risk in addition to TCD. Methods: We collected plasma samples from the peripheral blood of fifteen SCD patients (HbSS) at the time of either silent cerebral infarct (SCI) or stroke. Stroke (n=8) or SI was confirmed by MRI using diffusion imaging and T2 FLAIR. The cohort included 6 females and 9 males between the ages of 3 and 20 years. Plasma from fifteen age and gender matched control patients with HbSS but had normal MRIs and TCD velocities less than 170 m/s. We measured plasma levels of BDNF, MPO, VCAM-1 and ICAM-1 using antibody immobilized fluorescent beads (Millipore, Billerica, MA) and Luminex xMAP technology (Bio-Rad, Hercules, CA). Student's t-test was used to analyze the difference in plasma levels between the stroke and control groups. Results and Conclusions: BDNF levels were significantly higher in the stroke group than in the control group; 2978.2 ± 960.3 pg/ml compared to 2200.8 ± 758.4 pg/ml, p=0.005. Difference in MPO levels between the two groups approached significance; 36617.2 ± 14828.9 pg/ml compared to 29521.9 ± 7889.6 pg/ml, p=0.07. Difference in VCAM-1 levels also approached significance; 143997.0 ± 9963.8 pg/ml compared to 138126.9 ± 11902.0 pg/ml, p=0.08, but there was no significant difference in ICAM-1 levels. Only BDNF plasma levels positively correlated with the area and volume of the infarct, p=0.009. These results further support the assertion that BDNF is involved in the pathophysiology of cerebrovascular disease in SCD. It is possible that MPO and VCAM-1 may also play a role, and that a significant difference was not found due to sample size limitations. Plasma BDNF levels, and possibly MPO and VCAM-1 plasma levels, have potential as biomarkers for stroke risk to improve the positive predictive value of TCD velocities in patients with SCD, or to assess risk in patients unable to receive TCDs. Disclosures No relevant conflicts of interest to declare.

EVALUATION OF IRON OVERLOAD AND ADEQUACY OF PACKED RED BLOOD CELLS TRANSFUSION IN CHILDREN WITH THALASSEMIA MAJOR

Background: Transfusion therapy in patients with thalassemia major needs to address the common questions such as what should be the optimal hemoglobin (Hb) level for effective transfusion and how do transfusion requirements affect the success of iron chelation therapy. Objective: The objective of the study was to evaluate iron overload and adequacy of packed red blood cells (PRBCs) transfusion in children with thalassemia major along with the correlation of serum ferritin level with transfusional iron load and pre?transfusion Hb level. Materials and Methods: This single?center retroprospective observational study was carried out in thalassemia day care center of tertiary care hospital for 1 year over 32 transfusion?dependent ??thalassemic patients up to 18 years of age. Data including pre?transfusion hemoglobin level, number and volume of PRBC transfused, and serum ferritin level for 1 year were analyzed, and annual transfusion iron load was calculated. Correlation of serum ferritin level with transfusional iron load and pre?transfusion Hb level was determined using Pearson coefficient (r) and p-value. Results: The mean pre?transfusion hemoglobin level was 6.4±0.23 g%. The average number and volume of PRBCs transfused in a year were 12.5±3.02 and 112 ml/kg, respectively, with the average annual transfusional iron load of 121.3 mg/kg ±28.9 or 0.3 mg/kg/day. A significant positive (r=0.4184, p=0.017) correlation was observed between serum ferritin level and transfusional iron load with the mean serum ferritin level of 1744±604.6 ng/ml. Negative correlation was observed between serum ferritin and pre?transfusion Hb level (r=?0.2624 and p=0.1537). Conclusion: All patients were undertransfused and this undertransfusion further leads to more accumulation of iron in the body through increased absorption of dietary iron as a result of anemia and ineffective erythropoiesis.

Potential of transfusion therapy in surgical practice in patients with liver cirrhosis (review of the literature)

Currently, there are no clear indications for transfusion therapy in patients with liver cirrhosis. The article describes in detail the main clinical syndromes and pathophysiological mechanisms of chronic hepatitis, including the outcome of cirrhosis of the liver against the background of endotoxemia. The authors paid attention to the justification of the potential of transfusion therapy in this category of patients.

Hemolytic reaction in the washed salvaged blood of a patient with paroxysmal nocturnal hemoglobinuria

BackgroundIn patients with paroxysmal nocturnal hemoglobinuria (PNH), the membrane-attack complex (MAC) formed on red blood cells (RBCs) causes hemolysis due to the patient’s own activated complement system by an infection, inflammation, or surgical stress. The efficacy of transfusion therapy for patients with PNH has been documented, but no studies have focused on the perioperative use of salvaged autologous blood in patients with PNH.Case presentationA 71-year-old man underwent total hip replacement surgery. An autologous blood salvage device was put in place due to the large bleeding volume and the existence of an irregular antibody. The potassium concentration in the transfer bag of salvaged RBCs after the wash process was high at 6.2 mmol/L, although the washing generally removes > 90% of the potassium from the blood. This may have been caused by continued hemolysis even after the wash process. Once activated, the complement in patients with PNH forms the MAC on the RBCs, and the hemolytic reaction may not be stopped even with RBC washing.ConclusionsPacked RBCs, instead of salvaged autologous RBCs, should be used for transfusions in patients with PNH. The use of salvaged autologous RBCs in patients with PNH should be limited to critical situations, such as massive bleeding. Physicians should note that the hemolytic reaction may be present inside the transfer bag even after the wash process.

Pre-transfusion testing for Ebola virus disease patients in serious communicable infectious diseases hospitals in Tokyo: A cross-sectional study

BackgroundEbola virus disease (EVD) was endemic to Africa in 2014–2016. Supportive therapies have been shown to improve the outcome of EVD, and additional supportive therapy including blood transfusion therapy and external circulation could be needed in the event of a future global outbreak. However, pre-transfusion testing policies and guidelines have not yet been established in Japan. MethodsWe conducted a cross-sectional study of blood transfusion therapy for EVD patients at three designated hospitals for serious communicable diseases in Tokyo. In each hospital, we surveyed blood transfusion therapy policy, blood transfusion protocol, presence of a specialist in the department of transfusion medicine, facility capacity for pre-transfusion compatibility testing, and types of personal protective equipment available. ResultsOne hospital had a cross-matched compatible blood transfusion policy, one had a cross-matched compatible blood transfusion policy only when the patient's ABO and RhD type is previously known, and the third had not created a policy. Two hospitals had a department of transfusion medicine. These two hospitals had a special testing unit for serious communicable diseases, while the other had a portable unit for testing. There were no major differences noted in available personal protective equipment. ConclusionPolicies and protocols differ among hospitals. The choice of blood transfusion policy and pre-transfusion testing is largely dependent on equipment and human resources. Further discussion is required to develop national guidelines for blood transfusion therapy in patients with serious communicable diseases, including countermeasures against complications and ethical issues related to the safety of patients and healthcare workers.