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Despite the substantial transfusion requirements, there are few studies on the optimal transfusion strategy in pediatric patients undergoing hematopoietic stem cell transplantation (HSCT). Our study aimed to retrospectively analyze red blood cell (RBC) and platelet (PLT) transfusion practices during the first 100 days after HSCT at the pediatric hematology/oncology unit of our hospital between 2016 and 2019, due to a more restrictive approach adopted after 2016. We also evaluated the impact on patient outcomes. A total of 146 consecutive HSCT patients were analyzed. In patients without hemorrhagic complications, the Hb threshold for RBC transfusions decreased significantly from 2016 to 2017 (from 7.8 g/dL to 7.3 g/dL; p = 0.010), whereas it remained the same in 2017, 2018, and 2019 (7.3, 7.2, and 7.2 g/dL, respectively). Similarly, the PLT threshold decreased significantly from 2016 to 2017 (from 18,000 to 16,000/μL; p = 0.026) and further decreased in 2019 (15,000/μL). In patients without severe hemorrhagic complications, the number of RBC and PLT transfusions remained very low over time. No increase in 100-day and 180-day non-relapse mortality or adverse events was observed during the study period. No patient died due to hemorrhagic complications. Our preliminary observations support robust studies enrolling HSCT patients in patient blood management programs. [ABSTRACT FROM AUTHOR]

Background: Infants < 10 kg undergoing cardiac surgery with cardiopulmonary bypass (CPB) may receive either fresh frozen plasma (FFP) or other solutions in the CPB priming volume. The existing comparative studies are controversial. No study addressed the possibility of total avoidance of FFP throughout the whole perioperative course in this patient population. This retrospective, non-inferiority, propensity-matched study investigates an FFP-free strategy compared to an FFP-based strategy. Methods: Among patients <10 kg with available viscoelastic measurements, 18 patients who received a total FFP-free strategy were compared to 27 patients (1:1.5 propensity matching) receiving an FFP-based strategy. The primary endpoint was chest drain blood loss in the first 24 postoperative hours. The level of non-inferiority was settled at a difference of 5 mL/kg. Results: The 24-h chest drain blood loss difference between groups was −7.7 mL (95% confidence interval −20.8 to 5.3) in favor of the FFP-based group, and the non-inferiority hypothesis was rejected. The main difference in coagulation profile was a lower level of fibrinogen concentration and FIBTEM maximum clot firmness in the FFP-free group immediately after protamine, at the admission in the ICU and for 48 postoperative hours. No differences in transfusion of red blood cells or platelet concentrate were observed; patients in the FFP-free group did not receive FFP but required a larger dose of fibrinogen concentrate and prothrombin complex concentrate. Conclusions: An FFP-free strategy in infants < 10 kg operated with CPB is technically feasible but results in an early post-CPB coagulopathy that was not completely compensated with our bleeding management protocol. [ABSTRACT FROM AUTHOR]

A real-world analysis was conducted in Italy among sickle cell disease (SCD) patients to evaluate the epidemiology of SCD, describe patients' characteristics and the therapeutic and economic burden. A retrospective analysis of administrative databases of various Italian entities was carried out. All patients with ≥1 hospitalization with SCD diagnosis were included from 01/2010-12/2017 (up to 12/2018 for epidemiologic analysis). The index date corresponded to the first SCD diagnosis. In 2018, SCD incidence rate was 0.93/100,000, the prevalence was estimated at 13.1/100,000. Overall, 1816 patients were included. During the 1st year of follow-up, 50.7% of patients had one all-cause hospitalization, 27.8% had 2, 10.4% had 3, and 11.1% had ≥4. Over follow-up, 6.1–7.2% of patients were treated with SCD-specific, 58.4–69.4% with SCD-related, 60.7–71.3% with SCD-complications-related drugs. Mean annual number per patient of overall treatments was 14.9 ± 13.9, hospitalizations 1.1 ± 1.1, and out-patient services 5.3 ± 7.6. The total mean direct cost per patient was EUR 7918/year (EUR 2201 drugs, EUR 3320 hospitalizations, and EUR 2397 out-patient services). The results from this real-world analysis showed a high disease burden for SCD patients with multiple hospitalizations during the follow-up. High healthcare resource utilization and costs were associated with patient' management and were most likely underestimated since indirect costs and Emergency Room admissions were not included. [ABSTRACT FROM AUTHOR]

Background: Two million transfusions are performed in Spain every year. These come at a high economic price for the health system, increasing the morbidity and mortality rates. The way of obtaining the hemoglobin concentration value is via invasive and intermittent methods, the results of which take time to obtain. The drawbacks of this method mean that some transfusions are unnecessary. New continuous noninvasive hemoglobin measurement technology can save unnecessary transfusions. Methods: A prospective study was carried out with a historical control of two homogeneous groups. The control group used the traditional hemoglobin measurement methodology. The experimental group used the new continuous hemoglobin measurement technology. The difference was analyzed by comparing the transfused units of the groups. The economic savings was calculated by multiplying the cost of a transfusion by the difference in units, taking into account measurement costs. Results: The percentage of patients needing a transfusion decreased by 7.4%, and the number of transfused units per patient by 12.56%. Economic savings per patient were €20.59. At the national level, savings were estimated to be 13,500 transfusions (€1.736 million). Conclusions: Constant monitoring of the hemoglobin level significantly reduces the need for blood transfusions. By using this new measurement technology, health care facilities can significantly reduce costs and improve care quality. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Thalassemia is a quantitative disorder of haemoglobin production. It is a multisystem disorder which mainly affects the heart, liver, endocrine and bone and is treated with regular blood transfusions and chelation therapy. A number of recent studies have also reported alteration in pulmonary functions with this disorder which have been ascribed either to the disease or its therapy. Affected children frequently develop pulmonary complications and subsequently growth retardation. This study was undertaken to assess pulmonary function in thalassemic children who had received regular transfusions and chelation therapy. AIM: To study pulmonary function tests (PFT) in multitransfused children with thalassemia and compare them with normal children. MATERIALS AND METHODS: Pulmonary function tests were done on 35 children with βthalassemia major attending the thalassemia clinic of a teaching hospital over a period of 1 year. Serum ferritin and haemoglobin levels were also estimated in these children. The PFT was compared with 35 age and sex matched controls. Student's t test was used to compare the PFT parameters between these groups and the Pearson's correlation analysis was used to correlate various PFT parameters with serum ferritin, number of blood transfusion received and duration of chelation therapy. RESULTS: Mid peak expiratory flow (PEF) in thalassemic children was significantly lower than the control group (P=0.023). Among the thalassemic children, 27.71%, 14.28% and 2.85% had restrictive, obstructive and a mixed pattern of abnormalities respectively. In the study subjects, the assessed lung volumes (forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) and PEF, PEFR inversely correlated with serum Ferritin levels (r=0.23, 0.12, 0.09, respectively). Similarly, all measured lung volume parameters (FEV1, FVC, PEFR) also inversely correlated with cumulative blood transfusions given (r=0.20, 0.27, 0.276, respective- ly). Among the study group, children who were chelated early were found to have better lung functions (FEV1, FVC, PEFR) than those chelated late or not at all (r=0.27, 0.48, 0.25, respectively). This correlation was particularly significant between FVC and duration of chelation therapy (P=0.014) CONCLUSIONS: Our study found a significant reduction in lung volume parameters in chronically transfused thalassemia major patients. Iron overload and number of blood transfusions given correlated with deterioration in lung functions but early initiation of chelation therapy correlated well with better lung functions when compared to those in whom chelation was started late or not at all. [ABSTRACT FROM AUTHOR]