Pretransfusion Hemoglobin Level Research Articles

Magnetic Resonance Evaluation of Tissue Iron Deposition and Cardiac Function in Adult Regularly Transfused Thalassemia Intermedia Compared with Thalassemia Major Patients.

Objectives: This multicenter, retrospective, population-based, matched-cohort study compared clinical characteristics and magnetic resonance imaging (MRI) findings, including hepatic, pancreatic, and cardiac iron levels and cardiac function, between 135 adult regularly transfused thalassemia intermedia (TI) patients (44.73 ± 12.16 years, 77 females) and 135 age- and sex-matched thalassemia major (TM) patients (43.35 ± 9.83 years, 77 females), enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Methods: The MRI protocol included the quantification of hepatic, pancreatic, and cardiac iron levels (R2* technique), the assessment of biventricular function parameters (cine images), and the detection of replacement myocardial fibrosis (late gadolinium enhancement technique). Results: Age, sex, frequency of splenectomy and chelation, and serum ferritin levels were not significantly different (p > 0.05) between the two groups, but TI patients started regular transfusions significantly later (p < 0.0001) and showed significantly lower pre-transfusion hemoglobin levels (p = 0.005). No difference was found in hepatic iron levels (p = 0.853). TI patients exhibited significantly lower pancreatic R2* values (p < 0.0001), also correcting for the duration of regular transfusions, and significantly lower cardiac R2* values (p < 0.0001). In the receiver operating characteristic analysis, pancreatic iron was the strongest discriminator between the two diseases. Left and right ventricular end-diastolic volume indexes were significantly higher in TI than in TM patients (p = 0.003 and p = 0.046, respectively), but the correction for the duration of regular transfusions removed the disease-specific differences (p > 0.05). Left ventricular (LV) mass index was significantly higher in TI (p = 0.049), while no difference (p > 0.05) was found in biventricular ejection fractions and replacement myocardial fibrosis. Conclusions: TI patients showed lower pancreatic and cardiac iron burden and more pronounced LV hypertrophy. These differences could not be explained by the different duration of the transfusional regimen.

Impact of Patient Blood Management on Red Blood Cell Utilization in an Urban Community Teaching Hospital: A Seven-Year Retrospective Study.

This study aimed to showcase how implementing a patient blood management (PBM) program effectively cuts unnecessary red blood cell (RBC) transfusions in a New York City urban community teaching hospital. Analyzing seven years from 2013 to 2019, a retrospective review of RBC transfusions was conducted. Following the introduction of PBM, considerable improvements were observed annually. These included a drop in mean pretransfusion hemoglobin levels from 7.26 g/dL (2013) to 6.58 g/dL (2019), a 34% reduction in yearly RBC unit transfusions, and fewer units given to patients with pre-Hgb levels ≥ 7 g/dL (from 1210 units in 2013 to 310 units in 2019). Furthermore, this study noted a decline in two-unit RBC orders when Hgb levels were ≥ 7 g/dL from 65 orders in 2013 to merely 3 in 2019. The estimated total cost savings attributed to the six-year PBM program duration after full implementation in 2014 amounted to USD 2.1 million. Overall, PBM implementation significantly decreased RBC transfusions and enhanced transfusion practices. The findings emphasize that successful PBM strategies do not always necessitate extensive resources or increased budgets but instead rely on the application of intuitive methods, as evidenced by this study.

Pretransfusion hemoglobin level and mortality in adults with transfusion-dependent β-thalassemia

Regular transfusions have dramatically improved the outcomes for patients with thalassemia major, especially reducing deaths from anemia-related heart failure, but the hemoglobin threshold for maximizing outcomes is the subject of conjecture. Musallam et al report on a retrospective analysis of 10-year survival data in 779 patients, identifying increasing survival to be associated with higher pretransfusion hemoglobin levels, with the highest survival observed when levels were maintained &amp;gt;10.5 g/dL. These data support raising the target threshold for pretransfusion hemoglobin levels in patients with transfusion-dependent thalassemia.

Real-World Feasibility of RH Genotype-Matched Red Blood Cells for Chronically Transfused Patients with Sickle Cell Disease

Introduction Most red blood cell (RBC) alloimmunization in sickle cell disease (SCD) is caused by sensitization to Rh (D, C, c, E, and e) and K antigens. Provision of serologic Rh and K-matched RBCs has reduced but not eliminated Rh alloantibody formation due to the high genetic variation within the RH loci in Blacks. Over 85% of patients with SCD, as well as Black donors needed to support CEK matching programs, have variant RHD or RHCE alleles that result in loss or alteration of Rh antigens. These variant Rh proteins are not detected by standard serologic tests, and thus, genetically matched RBCs may be necessary to prevent all Rh alloimmunization events. We previously used bioinformatic modeling to show that RH genetic matching was achievable for a cohort of ~200 patients of whom ~50% were chronically transfused, and represented 98 different RHD/RHCE allele combinations, with only 10 additional donations each weekday (~1050 Black donors/month) than required for serologic Rh and K matching. The real-world feasibility of providing RH genotype-matched RBCs to patients requiring regular transfusion has not been tested. Methods We performed a pilot study to evaluate the feasibility of providing RH genotype-matched RBCs from Black and Hispanic donors to non-Rh- and Rh-alloimmunized chronically transfused patients with SCD (1 to 18 years old). Black donor units at the blood center were first screened by serology, HEA BeadChip, and in-house RH SNP assays, and those selected for transfusion to study subjects had comprehensive RHD and RHCE genotyping. Data captured included RH genotypes of matched units identified, transfusion delays, unit age at time of transfusion, and pre-transfusion hemoglobin (Hb) and HbS levels. On-study values were compared to pre-study values obtained in the 6 to 12 transfusion visits prior to study enrollment. Results The study opened in February 2020. Due to the COVID-19 pandemic, donor center collections dropped to a nadir of ~500 Black donor units/month in 2020 but returned to ~1500 units/month currently, similar to pre-COVID numbers. For 14 participants (13 Black, 1 Hispanic), we transfused 327 RH genotype-matched units; 287 (88%) were exact RH genotype-matched (donor with identical genotype, homozygous for a matching RH haplotype, or RH allele match) and 40 (12%) were RH matched with the provision that RHD*RHD and *DAU0 and RHCE*ce and *ce48C are equivalent since no epitopes appear to differ between these alleles. No transfusion visits (n=183) were postponed due to the inability to identify RH genotype-matched units, including during the pandemic and national blood shortages that had a disproportionate impact on Black donations. Using individual patients as factors, ANOVA showed no statistically significant difference in unit age (days, Figure) or pre-transfusion Hb values between pre- and on-study transfusion visits. Although the goal maintenance pre-transfusion HbS levels varied between 30 and 50%, a statistically significant lower mean HbS on- (n=183) vs pre-study (n=139) (ANOVA F-ratio 15.4, p=0.000) (Figure) potentially suggests improved transfused RBC survival with RH genotype matching. This feasibility trial was not powered to detect a difference in alloantibody formation, but no Rh nor non-Rh antibody have been detected. Conclusion Recruitment of minority blood donors is a growing initiative nationally needed to support CEK matching. Our preliminary results suggest that genetic RBC matching at the RH loci may be feasible with Black and Hispanic donors. Donor center RH genotyping can facilitate transfusion of RH genotype-matched RBCs, improve allocation of minority and rare donor units and, at the same time, potentially improve patient care by avoiding Rh alloimmunization for individuals with SCD.

Adults with Transfusion-Dependent Thalassemia Have Variable Clinical Profiles Depending on Where They Receive Care: Thalassemia Treatment Center Vs Community

Introduction: Advances in the treatment of transfusion-dependent thalassemia (TDT) have decreased complications and improved survival. Established TDT guidelines have improved outcomes through regular red cell transfusions to maintain a pre-transfusion hemoglobin &amp;gt; 9-9.5 g/dL and chelation to maintain a serum ferritin &amp;lt; 1500 ng/mL, liver iron content &amp;lt; 5 mg/g dry weight and a cardiac T2*&amp;gt; 20 ms. Increased lifespan of individuals with TDT now necessitates adult hematology providers with capacity to manage uncomplicated thalassemia. While it is recommended that thalassemia care be provided through a multidisciplinary thalassemia treatment center (TTC), there are a limited number of TTCs in the United States. As a result many adults with TDT receive care in the community from hematologists with variable knowledge and experience with thalassemia. In this study, we thus sought to evaluate the prior care of patients referred to an adult TTC in order to determine if there was a significant difference in the care provided by a pediatric TTC compared to community hematologists. Methods: We evaluated adult patients (&amp;gt; 18 years old) with a diagnosis of TDT referred to the Penn Comprehensive Adult Thalassemia Program. Patients were either referred from the Children's Hospital of Philadelphia TTC via a dedicated transition program, or from community hematologists (Comm). Data collection was performed via manual chart review. Data collected included the mean of three consecutive pre-transfusion hemoglobin levels prior to the initial visit, serum ferritin, and most recent liver and cardiac iron quantitation by MRI. Results: Of the 94 patients with thalassemia referred to the adult program between 2013 and 2023, 54 had a diagnosis of TDT. Twenty-seven patients were referred each from the TTC and the community. At the initial adult thalassemia center visit, the median age was 38.5 years (TTC) and 28 years (Comm). More patients in the TTC group maintained a pre-transfusion Hgb &amp;gt; 9 g/dL (78% vs 52%). Serum ferritin trended lower in the TTC group (1725 vs 2629 ng/ml, p-value = 0.056). While 67% of the TTC group had a serum ferritin &amp;lt; 1500 ug/mL compared to 41% in the Comm group, there was no significant difference in the liver iron content between both groups (TTC 10.2 vs Comm 12.2 mg/g dry weight). However, 60% of the TTC group compared to 48% in the Comm group had a LIC &amp;lt; 5. Those followed at the TTC possibly have more cardiac iron with 4 vs 1 patient in the TTC vs Comm group having a T2* &amp;lt; 20 ms, likely reflective of more complicated patients being referred to the TTC. Conclusion : Patients managed by a TTC were more likely to be adequately transfused with serum ferritins &amp;lt; 1500 ng/mL. Maintaining a pre-transfusion hemoglobin &amp;gt; 9-9.5 g/dL and a serum ferritin &amp;lt; 1500 ng/mL are known to reduce long-term complications of chronic anemia, ineffective erythropoiesis and iron overload in thalassemia. Thus in order to improve long-term outcomes in patients with TDT, we propose that all adult hematology providers, regardless of care setting should aim to gain knowledge on the management of individuals with thalassemia and establish collaborative care relationships with experts in thalassemia care.

Effect of flaxseed intervention on iron overload and its complications in thalassemia major patients

PurposePhytochemicals are known for their iron chelation ability with no side effects. Lignans and polyphenols in flaxseed have iron-chelating and antioxidant abilities. Therefore, this study aims to assess the ability of flaxseed to reduce iron overload and minimize its negative health effects.Design/methodology/approachA randomized control trial was conducted. A total of 96 patients were enrolled in this study and randomized into three groups: two intervention groups had 3 or 7 g of whole flaxseed and a control group took a placebo (gram seeds). Interventions were administered for 12 weeks. A hematological profile, ferritin quantification in serum, and liver function tests were performed to evaluate the effects of flaxseed.FindingsIt was observed that flaxseed with synthetic iron chelators caused a significant reduction in ferritin levels compared with synthetic chelators. The pre-transfusion hemoglobin level was also improved in the intervention group. A significant reduction (p value 0.0003) in ferritin levels among intervention groups indicated that flaxseed reduced the iron overload. It can be concluded that flaxseed reduced iron overload in thalassemia major patients with no side effects and may reduce iron overload solely when taken in adequate quantity.Originality/valueAlthough the study targets a non-specific protein-ferritin for the evaluation of iron-overload, the initial ferritin value and reduction in these levels indicate the efficacy of ferritin for reducing iron overload in thalassemia major patients. Moreover, this is the first study, to the best of the authors’ knowledge, that demonstrates the iron-chelation ability of flaxseed in thalassemia major patients, suggesting the use of flaxseed for iron chelation.

Pattern and clinical correlates of renal iron deposition in adult beta-thalassemia major patients.

We evaluated pattern and clinical correlates of renal T2* measurements in adult β-thalassemia major (β-TM) patients. Ninety β-TM patients (48 females, 38.15 ± 7.94years), consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia network, underwent T2* magnetic resonance imaging (MRI) for quantification of iron overload (IO) in kidneys, liver, pancreas, and heart. Ten (11.1%) patients showed renal IO (T2* < 31ms). Global kidney T2* values did not show a correlation with gender, age, splenectomy, regular transfusions or chelation starting age, pre-transfusion hemoglobin, and serum ferritin levels. Global kidney T2* values showed an inverse correlation with MRI liver iron concentration (LIC) values (R = - 0.349; p = 0.001) and a positive correlation with global pancreas T2* values (R = 0.212; p = 0.045). Frequency of renal IO was significantly higher in patients with cardiac IO than in patients without cardiac IO (50.0% vs. 6.3%; p = 0.001). A significant inverse association was detected between global kidneys T2* values and lactate dehydrogenase (LDH) (R = - 0.529; p < 0.0001). In multivariate regression analysis, MRI LIC and LDH were the strongest predictors of global kidney T2* values. A MRI LIC > 4.83mg/g dw predicted the presence of renal IO (sensitivity = 90.0%; specificity = 61.2%). Global kidney T2* values were inversely correlated with uric acid (R = - 0.269; p = 0.025). In conclusion, in adult β-TM patients, renal iron deposition is not common and is linked to both hemolysis and total body iron overload.

To study the Transfusion Practice with respect to Component Therapy in Major Obstetric Hemorrhage

Introduction: Peripartum hemorrhage is a major complicationrequiring emergency transfusion of packed blood or itscomponents with quick decision-making. We describeour experience of transfusion practices in major obstetrichemorrhage.Materials and Methods: Retrospective observational dataof all the deliveries (n = 1837) over period of one year.All cases were assessed for obstetric hemorrhage andsubsequent transfusion practices done. Demographic details,obstetric history, indications for transfusion, gestational ageat transfusion, pre- and post-transfusion hemoglobin levels,nature and type of transfusion, units of transfusion, adverseevents following transfusion and outcome in terms of morbidityand mortality were noted. Data was analyzed using SPSS 21.0software. Chi-square test was used for analysis.Results: Transfusion was required in 776 (42.2%) pregnantwomen. Obstetric hemorrhage was the indication in 432 (55.7%)of the women receiving a transfusion. Other indications wereanemia during pregnancy (39.7%) and traumatic pph (10.5%),respectively. Among major obstetric hemorrhage cases (n =432), the majority (n = 224; 51.9%) had antepartum hemorrhage(abruptio placentae/placenta previa) and the remaining 208(48.1%) had postpartum hemorrhage change no significantchange seen. A total of 138/432 (31.9%) cases had pretransfusionhemoglobin level &lt;8 g/dl in cases of moderateanemia. PRBC was transfused to 319 (73.8%) followed byplatelets (212; 49.1%) and FFP (n = 29; 6.7%), respectively.The majority of cases required only 2 units of transfusion.There were 21 (4.9%) cases requiring more than 3 units.Post-transfusion hemoglobin levels were &gt;10 g/dl in 319/432(73.8%) cases, thus showing a significant change (p &lt; 0.05).Most common adverse reactions (fever/shivering) were notedin 32 (7.4%) cases. There were 6 (1.4%) mortalities (all havingpre-transfusion Hb &lt;6 g/dl).Conclusion: The study highlighted the significance of packedblood cells and component transfusion in reducing obstetricmorbidity and mortality

Safety of hypoxic red blood cell administration in patients with transfusion-dependent hematological malignancies: An interim analysis

Anemia is a common symptom of hematological malignancies and red blood cell (RBC) transfusion is the primary supportive treatment, with many patients becoming transfusion dependent. Hemanext Inc. (Lexington, MA, United States) has developed a CE mark certified device to process and store RBCs hypoxically – citrate-phosphatedextrose (CPD)/phosphate-adenine-glucose-guanosine-saline-mannitol (PAGGSM) RBCs, leukocytes-reduced (LR), O2/CO2 reduced – with the aim of improving RBC quality for transfusion. This interim analysis describes the first patients to receive hypoxic RBCs, administered as part of a pilot post-marketing study in Norway. The primary outcome was adverse events (AEs) within 24 h of transfusion initiation and overall up to 7 days ( ± 1 day) post-transfusion. Secondary outcomes included changes in hemoglobin levels post-transfusion. Five patients with hematological malignancies were included (80 % male, mean age 69.8 [SD ± 19.3] years). Prior to the study, patients had been receiving conventional RBC transfusions every two weeks. Patients received 2 units of hypoxic RBCs over 2 h without complication. One mild AE (rhinovirus) was reported two days post-treatment and was deemed unrelated to treatment. The mean ± SD pre-transfusion hemoglobin level was 7.7 ± 0.5 g/dL, evolving to 9.0 ± 0.9 g/dL following administration of hypoxic RBCs; an increase of 17 %. This interim analysis showed that transfusion with hypoxic RBCs processed with the CPD/PAGGSM LR, O2/CO2 reduced system was effective and well tolerated in patients with hematologic malignancies. The overall clinical program will assess whether the use of hypoxic RBCs can reduce transfusion interval versus conventional RBCs in patients requiring acute and chronic transfusions.

5574716 PROBLEMS AND STRATEGIES OF BLOOD TRANSFUSION IN THALASSEMIA DURING COVID-19 PANDEMIC IN INDONESIA

Transfusion-dependent thalassemia is the most severe form of thalassemia in which patients require a regular blood transfusion to maintain their haemoglobin level. COVID-19 pandemic has disrupted the routine measure in controlling chronic diseases like thalassemia. This study aims to measure the difference in pre-transfusion haemoglobin level and frequency of transfusion before and during pandemics. This retrospective cross-sectional study utilized medical records data of 101 transfusion-dependent thalassemia (TDT) patients treated in Cipto Mangunkusumo Hospital (CMH) from 2019-2021. The dependent variables of this study were pre-transfusion haemoglobin level and transfusion attendance. The pre-pandemic phase was defined from March 30, 2019, to March 29, 2020, whereas the during-pandemic phase was from March 30, 2020, to March 29, 2021. Up to 59.4% of subjects had suboptimal Hb level of < 9.0 g/dL even before the pandemic and it increased to 71.3% during a pandemic. Transfusion frequency of pre-pandemic and during-pandemic phases showed no significant difference (p-value = 0.990). The mean pre-transfusion haemoglobin level before the pandemic was 8.71 g/dL and it decreased to 8.46 g/dL (p-value <0.001). Our study showed poorer control of pre-transfusion Hb levels during the pandemic and decreased transfusion frequency. This puts them at a higher risk of developing many long-term complications.

5588005 IMPACT OF COVID-19 PANDEMIC TO PRE-TRANSFUSION HEMOGLOBIN LEVEL AND FREQUENCY OF TRANSFUSION IN TRANSFUSION-DEPENDENT THALASSEMIA PATIENTS IN INDONESIA

Introduction: Transfusion-dependent thalassemia is the most severe form of thalassemia in which patients require regular blood transfusion to maintain their hemoglobin level. COVID-19 pandemic has disrupted the routine measure in controlling chronic diseases like thalassemia. Aims: This study aims to measure the difference in pre-transfusion hemoglobin level and frequency of transfusion before and during pandemic. Methods: This retrospective cross-sectional study utilized medical records data of 101 transfusion dependent thalassemia beta (TDT) patients treated in Cipto Mangunkusumo Hospital (CMH) from 2019-2021. Dependent variables of this study were pre-transfusion hemoglobin level and transfusion attendance. Pre-pandemic phase was defined from March 30, 2019 to March 29, 2020, whereas during-pandemic phase was from March 30, 2020 to March 29, 2021. Results: Up to 59.4% of subjects had suboptimal Hb level of < 9.0 g/dL even before the pandemic and it increased to 71.3% during pandemic. Transfusion frequency of pre-pandemic and during-pandemic phase showed no significant difference (p value = 0.990). The mean pre-transfusion hemoglobin level before pandemic was 8.70 g/dL and it decreased to 8.45 g/dL (p value <0.001). Summary - Conclusion: Our study showed poorer control of pre-transfusion Hb level during the pandemic and decreased transfusion frequency. This puts them at higher risk of developing many long-term complications.

Effect of single-unit transfusion in patients treated for haematological disease including acute leukemia: A multicenter randomized controlled clinical trial

BackgroundRetrospective studies in hematological unit have suggested that single red blood cell (1-RBC) unit transfusion policy may reduce the number of RBC used without negative clinical impact. MethodAcute leukemia patients requiring intensive chemotherapy or patients receiving autologous or allogeneic transplantation were randomly assigned to receive either single RBC (1-RBC arm) or double RBC (2-RBC arm) per transfusion with a hemoglobin trigger of 8 g/dL. The primary composite endpoint was the percentage of patients experiencing serious complications, such as a non-hematological adverse event grade ≥ 3 or intensive care admission or death. FindingsA total of 981 and 592 RBC transfusions were required in the 1-RBC arm (n = 125) and the 2-RBC arm (n = 120), respectively. The mean pre-transfusion hemoglobin levels were 7.49 ± 0.83 g/dL in the 1-RBC arm and 7.46 ± 0.67 g/dL in the 2-RBC arm (p = 0.275). The predefined non-inferiority criteria was achieved with 28/125 patients reaching the primary endpoint in the 1-RBC arm (22.4 %) and 28/120 patients in the 2-RBC arm (23.3 %) (Risk difference 0.009; 95 %, Confidence interval [−0.0791 to 0.0978], p = 0.021). The median (IQR) of RBC units transfused per patient was 7 (4–12) in the 1-RBC arm and 8 (4–12) in 2-RBC arm. Hemoglobin levels at discharge were also comparable in both arms. InterpretationThe results of this trial indicate that a single RBC transfusion policy is not inferior to a double RBC transfusion policy for patients receiving a bone marrow transplant or intensive chemotherapy in a hematological intensive care unit. However, the single RBC transfusion policy did not reduce the number of RBC units transfused per stay. FundingThis trial was funded by a grant from the French Ministry of Health

Blood donor, component, and recipient-specific factors associated with venous thromboembolism in transfused hospitalized adult patients: Data from the recipient epidemiology and donor evaluation Study-III (REDS-III).

Growing evidence suggests multiple pathophysiological mechanisms linking red blood cells (RBC) transfusions to thrombosis. This study examined blood donor, component, and recipient factors which may be associated with thromboembolic outcomes following RBC transfusion. We utilized the Recipient Epidemiology Donor Evaluation Study-III (REDS-III) database on patients transfused in 12 hospitals between 2013-2016. Stratified Cox proportional hazards regression models with time-dependent exposures were used to examine associations of donor and component modification characteristics on venous thromboembolism (VTE) in patients transfused RBC units. 59,603 patients were transfused 229,500 RBC units during 79,298 hospitalizations with post-transfusion VTE occurring in 1869 (2.4%) of patients. In adjusted regression analyses, a per RBC-unit risk of VTE was present for gamma irradiation (HR=1.03; 95% CI: 1.02-1.03), female donor sex (HR=1.01; 95% CI: 1.00-1.01), storage duration greater than 5 weeks (HR=1.01; 95% CI: 1.01-1.02), AS-1 storage solution (HR=1.01; 95% CI: 1.00-1.01), and apheresis-derived collections (HR=1.01; 95% CI: 1.01-1.02). Among recipient factors, male sex (HR=1.03; 95% CI: 1.02-1.04), pre-transfusion hemoglobin level (HR=0.94; 95% CI: 0.94-0.94), body mass index strata (HR=1.11; 95% CI: 1.08-1.14), and principal diagnoses including malignancy (HR=1.13; 95% CI: 1.10-1.16), cardiac arrest (HR=1.38; 95% CI:1.07-1.77) and hip fracture (HR=1.59; 95% CI:1.53-1.66) were associated with VTE in adjusted analyses. We identified several donor, component, and recipient-specific factors associated with VTE in transfused hospitalized adult patients. In adjusted models, the dose-dependent associations of donor and component-specific factors with VTE were modest and unlikely to be clinically significant in the majority of transfused patients. Additional mechanistic and clinical studies linking blood donor and component factors with thrombotic outcomes are needed.

PERBEDAAN KADAR HEMOGLOBIN PRATRANSFUSI PADA PASIEN TALASEMIA BETA MAYOR SEBELUM DAN SAAT PANDEMI COVID-19

Beta thalassemia major is a disorder that occurs due to the absence of beta globin chain production and is characterized by severe clinical symptoms. The purpose of this study was to explain the differences in pretransfusion hemoglobin levels in beta thalassemia major patients before and during the COVID-19 pandemic at Idaman Hospital Banjarbaru in 2019-2020. This research was a descriptive-analytic study with a cross sectional approach. Collecting data through medical records of patients with beta thalassemia major. The sampling technique was total sampling. The sample was 35 patients. The results showed that there were 18 women (51,43%) patients, the most age was in the age range 12 - &lt;18 as many as 12 (34,29%) patients, and underweight nutritional status (BB/TB) were 24 (68,57) patients. Pre-transfusion hemoglobin levels were classified into before and during the COVID-19 pandemic. The average value of pretransfusion hemoglobin levels before the COVID-19 pandemic was 8,95 g/dL and during the COVID-19 pandemic was 8,92 g/dL. The test used was the paired sample test and the results obtained were p = 0,83 because p &gt; 0,05, there was no significant difference between pretransfusion hemoglobin levels before and during the COVID-19 pandemic

Restrictive effects of thalassemia on respiratory functions: One center experience.

Respiratory functions in thalassemia major (TM) patients concerning poor chelation are a frequently researched issue. Our study aims to evaluate the lung functions of our patients with TM in the chronic transfusion program and to correlate them with their age, ferritin levels, and pre-transfusion hemoglobin values. Height, weight, pulmonary function test (PFT) results, pre-transfusion hemoglobin levels, and ferritin levels of 97 patients (55 boys and 42 girls) without any underlying cardiac or chronic respiratory disease were recorded. PFT is consisted of forced vital capacity (FVC) and forced expiratory volume in one second (FEV1), the ratio of FEV1/FVC to peak expiratory flow (PEF), and forced mid-exhaled flow between 25% and 75% of mid-expiratory flow (MEF25-75). Data were analyzed with IBM SPSS V25. Low FVC was observed in 58 patients (60%), and low FEV1 was observed in 26 patients (27.6%). Low PEF was observed in 62 patients (64.5%), and low MEF25-75 was observed in 8 (8.3%). PFT was affected in 75 patients (78.1%). The pattern of involvement was restrictive. Age, height, and ferritin values significantly affected the MEF25-75 (p<0.05). Age and pre-transfusion hemoglobin values had a significant effect on the FVC test (p<0.05). There was a weak negative correlation between ferritin values and MEF25-75 (r=-0.221) and a weak positive correlation between pre-transfusion hemoglobin and FVC (r=0.222). Age and height are the main risk factors affecting FEV1, MEF25-75, and PEF. Serum ferritin has only an effect on MEF25-75 in our study. The respiratory functions of TM patients were affected in a restrictive pattern.

Impact of COVID-19 Pandemic on Pre-Transfusion Hemoglobin Level and Frequency of Transfusion in Transfusion-Dependent Thalassemia Patients in Indonesia

Transfusion-dependent thalassemia is the most severe form of thalassemia; patients require regular blood transfusions to maintain their hemoglobin level. The COVID-19 pandemic has disrupted the routine measures for controlling chronic diseases like thalassemia. This study aims to measure the difference in pre-transfusion hemoglobin levels and the frequency of transfusions before and during pandemic. This retrospective cross-sectional study utilized medical record data of 101 transfusion-dependent thalassemia (TDT) patients treated in Cipto Mangunkusumo Hospital (CMH) from 2019–2021. The dependent variables of this study were pre-transfusion hemoglobin level and transfusion attendance. The pre-pandemic phase was defined as 30 March 2019 to 29 March 2020, whereas the during-pandemic phase was from 30 March 2020 to 29 March 2021. Up to 59.4% of subjects had suboptimal Hb levels of &lt;9.0 g/dL, even before the pandemic, and this increased to 71.3% during the pandemic. The mean pre-transfusion hemoglobin level before the pandemic was 8.71 g/dL, and this decreased to 8.46 g/dL (p value &lt; 0.001). Transfusion attendance before and during the pandemic showed no significant difference (p-value = 0.990). Our study shows poorer control of pre-transfusion Hb levels during the pandemic. This puts patients at higher risk of developing many long-term complications.

Role of Thromboelastogram in monitoring the activation of the coagulation pathway and assessing the associated risk factors for hypercoagulable state in transfusion dependent thalassemia patients

BackgroundThromboembolic events are rare but one of the fatal complications in thalassemia. Assessment of the hypercoagulable state is not done regularly, and we have assessed the utility of Thromboelastography (TEG) for monitoring the activation of the coagulation pathway in patients with thalassemia. MethodologyA prospective single-center cohort study was conducted in a tertiary care set-up. Transfusion Dependent Thalassemia patients registered with the pediatric unit were screened for hypercoagulability using TEG during six months of the study period and followed up for three years for the development of thromboembolic events. Patient demographics, history of splenectomy, Serum ferritin levels and annual red cell transfusion requirement (mL/kg/year) were assessed. TEG parameters used were R time, K time, alpha angle, Maximum amplitude, Clot index, and Lysis 30. The thrombin generation test (V Curve) obtained from the first-degree derivate of the TEG velocity curve was also used for analysis. ResultsA total of 34 patients were recruited during the six months study period with an average age of 10.6 years ( ± 5.47). The average pre-transfusion hemoglobin level and the volume of packed red cells received were 7.24 g/dL and 152.82 mL/kg/year respectively. The TEG tracing was suggestive of a hypercoagulable state in 58.82% of patients. The mean values of angle (70.74), MA (64.16), CI (2.65) and TG (774.43) in TDT patients compared to age matched reference range (62.81, 57.99, 0.8, 577.83 respectively) was suggestive of prothrombotic changes. Annual blood transfusion requirement was negatively correlated with hypercoagulable status (‐0.344, CI= -0.68 to 0.08). One out of 34 patients developed corona radiata infarct (with annual blood requirement; 112.7 mL/kg/Year). The risk to develop a hypercoagulable state appeared to be higher when the volume of RBCs transfused was less than 154 mL/kg/Year. ConclusionTDT patients are at risk of developing thromboembolism, and screening with TEG may be useful to identify those at high risk.

The role of MRI-R2* in the detection of subclinical pancreatic iron loading among transfusion-dependent sickle cell disease patients and correlation with hepatic and cardiac iron loading

ObjectivesPancreatic reserve could be preserved by early assessment of pancreatic iron overload among transfusion-dependent sickle cell disease (SCD) patients. This study aimed to measure pancreatic iron load and correlate its value with patients’ laboratory and radiological markers of iron overload.Materials and methodsSixty-six SCD children and young adults underwent MRI T2* relaxometry using a simple mathematical spreadsheet and laboratory assessment.ResultsThe results indicated moderate-to-severe hepatic iron overload among 65.2% of studied cases. None had cardiac iron overload. Normal-to-mild iron overload was present in the pancreas in 86% of cases, and 50% had elevated serum ferritin > 2500 ug/L. There was no significant correlation between pancreatic R2* level, serum ferritin, and hepatic iron overload. Patients with higher levels of hemolysis markers and lower pre-transfusion hemoglobin levels showed moderate-to-severe pancreatic iron overload.ConclusionChronically transfused patients with SCD have a high frequency of iron overload complications including pancreatic iron deposition, thereby necessitating proper monitoring of the body’s overall iron balance as well as detection of extrahepatic iron depositions.

Passive order auditing associated with reductions in red blood cell utilization: National blood shortage experience.

Decreased blood collection during the Coronavirus Disease 2019 (COVID-19) pandemic resulted in long-term red blood cell (RBC) shortages in the United States. In an effort to conserve RBCs, the existing passive alert system for auditing inpatient transfusions was modified to activate at a lower hemoglobin threshold (6.5g/dL instead of 7.0g/dL for stable, nonbleeding inpatients) during a 9-month shortage at an academic medical center. Hemoglobin levels prior to RBC transfusions were compared for inpatients receiving RBC transfusions to determine whether RBC utilization changed during the intervention. This retrospective study compared the number of single-unit RBC transfusions and hemoglobin levels prior to RBC transfusion among inpatients during the 9months of the intervention (Period 2, 06/01/2021-2/28/2022) to the same period of the previous year (Period 1, 06/01/2020-2/28/2021). Overall full unit RBC transfusions to inpatients decreased by 15% from 5182 to 4421. Of all transfusions, 50.3% and 49.8% were single-unit RBC transfusions in Period 1 and Period 2, respectively. The incidence rate difference and incidence rate ratio of single RBC units transfused per 1000 patient days were significantly decreased (p=0.0007). The average pre-transfusion hemoglobin level significantly decreased from 7.18 g/dL to 7.05 g/dL (p=0.0002), largely due to significant decreases in hemoglobin transfusion triggers for adult inpatient ward transfusions. Modification of the passive alert system was associated with significantly decreased RBC utilization during a long-term RBC shortage. Modification of transfusion criteria recommended by passive alerts may be a feasible option to decrease RBC utilization at centers during long-term RBC shortages.

Standardizing blood dose using body surface area and analyze the effect of blood storage on hemoglobin increment within pediatric patients

Introduction: Pediatric patients exhibit a wide variation in weight which results in diverse transfusion practices. This study aims to standardize red blood cell (RBC) doses according to body surface area (BSA) and to analyze the role of RBC storage in post-transfusion hemoglobin levels. Material and methods: In this original prospective cohort study on hospitalized pediatric patients aged up to 14, we classified patients into transfusion-dependent (n = 31) and non-transfusion-dependent (n = 158). The non-transfusiondependent group was further classified into ≤10 kg (n = 72) or &gt;10 kg (n = 86) according to body weight (bw). We derived a regression equation between BSA and blood dose in non-transfusion-dependent subjects, and modified the equation by fixing blood dose to 15 mL/kg bw for only BSA based blood dose. We measured pre-transfusion and post-transfusion hemoglobin (Hb) levels, and ascertained effects of blood storage ≤15 days (n = 15) and &gt;15 days (n = 16) on posttransfusion Hb. Results: Pediatric patients ≤10 kg and &gt;10 kg bw (n = 158); mean ± standard deviation of weight and BSA were 4.5 ± 3.1 kg; 22.9 ± 10.4 kg; 0.26 ± 0.14 m 2 ; 0.9 ± 0.28) m 2 respectively. The regression equation ≤10 kg and &gt;10 kg bw when adjusted. Blood dose fixed at 15 mL/kg bw adjusted blood dose ≤10 kg; 15 mL/kg bw = −19.12 + 329.69x BSA m 2 . The regression equation &gt;10 kg bw: adjusted blood dose &gt;10 kg bw; 15 mL/kg bw = −158.8 + 563.3x BSA (m 2 ). The adjusted blood dose with BSA did not exceed 20 mL/kg bw. No significant differences were observed in pre and post-transfusion Hb in transfusion-dependent (n = 31) versus non-transfusion-dependent patients (n = 158) due to the RBC storage duration. Conclusions: RBC blood dose can be standardized by regression equation between standardized RBC dosage and BSA. Post-transfusion Hb is not dependent on days of RBC storage at the blood bank.