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Transfusion and Apheresis Science :... Oct 2023Sickle cell disease (SCD) is the most common hereditary hemoglobinopathy. The underlying pathophysiology of the red blood cell (RBC) leads to pan-systemic complications... (Review)
Review
Sickle cell disease (SCD) is the most common hereditary hemoglobinopathy. The underlying pathophysiology of the red blood cell (RBC) leads to pan-systemic complications which manifest at an early age. While curative and disease-modifying treatments exist for SCD, a key intervention in the management and treatment of SCD is RBC transfusion, which can alleviate or prevent many complications. SCD patients often require chronic RBC transfusion therapy which can result in complications, such as iron overload, alloimmunization and infection. In low- and middle-income countries (LMICs), SCD patients lack appropriate access to healthcare such as newborn screening, health education, prophylaxis for infection, and treatments to reduce both mortality and SCD-related adverse effects. Poor access to RBCs for transfusion, coupled with donated blood not meeting safety standards set by the World Health Organization, presents a significant barrier for patients requiring chronic transfusions in LMICs. Unmet needs associated with blood collection, blood component processing and recipient matching all pose a serious problem in many LMICs, although this varies depending on geographic location, political organizations and economy. This review aims to provide an overview of the global burden of SCD, focusing on the availability of current treatments and the burden of chronic RBC transfusions in patients with SCD.
Topics: Infant, Newborn; Humans; Anemia, Sickle Cell; Blood Transfusion; Erythrocytes; Erythrocyte Transfusion; Blood Group Incompatibility
PubMed: 37541800
DOI: 10.1016/j.transci.2023.103764 -
Pediatrics and Neonatology Jan 2024In newborns with hemolysis, the direct antiglobulin test (DAT) and indirect antiglobulin test (IAT) play a key role in demonstrating the presence of an immune cause. We...
BACKGROUND
In newborns with hemolysis, the direct antiglobulin test (DAT) and indirect antiglobulin test (IAT) play a key role in demonstrating the presence of an immune cause. We aimed to emphasize the importance of IAT in mothers of DAT-positive babies.
METHODS
DAT was performed with forward blood grouping on cord blood in term babies who were born between September 2020 and September 2022. IAT was performed in the mothers of the babies who were found to have a positive DAT and antibody identification was performed in the mothers who were found to have a positive IAT. Specific antibodies detected and identified were associated with the clinical course.
RESULTS
The study included 2769 babies and their mothers. The prevalence of DAT positivity was found to be 3.3% (87 of 2661). In DAT-positive babies, the rate of ABO incompatibility was 45.9%, the rate of RhD incompatibility was 5.7% and the rate of RhD and ABO incompatibility in association was 10.3%. The rate of subgroup incompatibility and other red blood cell antibodies was 18.3%. Phototherapy was applied because of indirect hyperbilirubinemia in 16.6% of the DAT-negative babies and in 51.5% of the DAT-positive babies. The need for phototherapy was significantly higher in DAT-positive infants (p < 0.01). Severe hemolytic disease of the newborn, bilirubin level, duration of phototherapy and use of intravenous immunoglobulin were found to be significantly higher in the babies whose mothers were IAT positive compared with the babies whose mothers were IAT negative (p < 0.01).
CONCLUSIONS
IAT should be performed on all pregnant women. When screening with IAT is not performed during pregnancy, performing DAT in the baby plays a key role. We showed that the clinical course was more severe when mothers of DAT-positive babies were IAT positive.
Topics: Infant; Infant, Newborn; Humans; Female; Pregnancy; Coombs Test; Retrospective Studies; Erythroblastosis, Fetal; Blood Group Incompatibility; Antibodies; Disease Progression; ABO Blood-Group System
PubMed: 37414722
DOI: 10.1016/j.pedneo.2023.05.001 -
Infection & Chemotherapy Jun 2023Neonatal jaundice is commonly seen in term and preterm newly born babies. It could be either physiologic or secondary to multiple underlying pathologies like urinary...
BACKGROUND
Neonatal jaundice is commonly seen in term and preterm newly born babies. It could be either physiologic or secondary to multiple underlying pathologies like urinary tract infection (UTI). Our main objective was to confirm the relationship between neonatal jaundice without apparent cause like hemolysis and the presence of UTI.
MATERIALS AND METHODS
We, retrospectively over a period extended from 2017 to 2020, included 496 babies admitted for elevated indirect hyperbilirubinemia for whom demographic, clinical, laboratory, and therapeutic data were collected through a detailed questionnaire.
RESULTS
Our study included 496 neonates and showed a prevalence of UTI in 8.9% of neonates. The two most common microorganisms in urine culture were (65.9%) and (25.0%). A multivariate logistic analysis showed that UTI was associated with male neonates (odds ratio [OR] = 2.366, 95% confidence interval [CI]: 1.173 - 4.774; = 0.016), history of prenatal UTI (OR = 5.378, 95% CI: 2.369 - 12.209; <0.001), poor feeding (OR = 3.687, 95% CI: 1.570 - 8.661; = 0.003), and positive urine culture in catheter (OR = 2.704, 95% CI: 1.255 - 5.826; = 0.011). The mean length of stay was higher in patients with positive UTI (Median = 216 hours) compared to patients with negative UTI (Median = 48 hours) ( <0.001).
CONCLUSION
Neonatal sreening for UTI should be recommended whenever there is unexplaind early or prolonged hyperbilirubinemia with no evidence of alloimmune hemolysis or blood group incompatibility and to prevent the morbidity of urosepsis and congenital kidneys malformations.
PubMed: 37407240
DOI: 10.3947/ic.2022.0117 -
Heliyon Jun 2023Blood transfusion; is considered an organ transplant. In coronary bypass surgery, large volumes of homologous blood transfusion may be required due to excessive...
PURPOSE
Blood transfusion; is considered an organ transplant. In coronary bypass surgery, large volumes of homologous blood transfusion may be required due to excessive bleeding. The large number of use of homologous blood transfusion in open heart surgery and the awareness of its various harmful effects have prompted researchers to conduct research on the use of autologous blood. With autologous transfusion, blood diseases, incompatibility, immunosuppression and organ damage can be prevented and the patient can be extubated earlier in the postoperative period.
METHODS
Between January 2020 and January 2016, a total of 176 patients, 56 in the treatment group (with autologous blood transfusion) and 120 in the control group, whose information could be reached from hospital records were investigated retrospectively.
RESULTS
No statistical difference was found between the mean intubation SO2 and PO2 values of the groups. On the contrary, considering the mean intubation times in the intensive care unit of both groups, the patients who underwent autologous blood transfusion were extubated at a statistically significant earlier time.
CONCLUSION
Autologous blood transfusion is a safe method in selected patients as well. Thanks to this method, patients are protected from complications associated with homologous blood transfusion. It is believed that performing autologous blood transfusion in selected patients undergoing open-heart surgery can decrease the number of postoperative transfusions, frequency of transfusion-related complications (especially in the lungs), and mean intubation times.
PubMed: 37383208
DOI: 10.1016/j.heliyon.2023.e17371 -
Fetal Diagnosis and Therapy 2023Targeted routine antenatal prophylaxis with anti-D immunoglobulin (Ig) only to RhD-negative pregnant women who carry RhD-positive fetuses (determined by fetal RHD...
INTRODUCTION
Targeted routine antenatal prophylaxis with anti-D immunoglobulin (Ig) only to RhD-negative pregnant women who carry RhD-positive fetuses (determined by fetal RHD genotyping) has reduced D-alloimmunization significantly when administered in addition to postnatal prophylaxis. Achieving high analysis sensitivity and few false-negative fetal RHD results will make RhD typing of the newborn redundant. Postnatal prophylaxis can then be given based on the result of fetal RHD genotyping. Terminating routine RhD typing of the newborns in cord blood will streamline maternity care. Accordingly, we compared the results of fetal RHD genotyping with RhD typing of the newborns.
METHODS
Fetal RHD genotyping was performed, and antenatal anti-D Ig was administered at gestational week 24 and 28, respectively. Data for 2017-2020 are reported.
RESULTS
Ten laboratories reported 18,536 fetal RHD genotypings, and 16,378 RhD typing results of newborns. We found 46 false-positive (0.28%) and seven false-negative (0.04%) results. Sensitivity of the assays was 99.93%, while specificity was 99.24%.
CONCLUSION
Few false-negative results support the good analysis quality of fetal RHD genotyping. Routine cord blood RhD typing will therefore be discontinued nationwide and postnatal anti-D Ig will now be given based on the result of fetal RHD genotyping.
Topics: Pregnancy; Female; Humans; Infant, Newborn; Prenatal Diagnosis; Fetal Blood; Genotype; Rh Isoimmunization; Rh-Hr Blood-Group System; Maternal Health Services; Fetus; Rho(D) Immune Globulin
PubMed: 37379821
DOI: 10.1159/000531694 -
The Journal of Extra-corporeal... Jun 2023ABOi heart transplant has become routine for the majority of children <2 years old. An 8-month-old child with complex congenital heart disease presented to the Medical...
BACKGROUND
ABOi heart transplant has become routine for the majority of children <2 years old. An 8-month-old child with complex congenital heart disease presented to the Medical University of South Carolina Shawn Jenkins Children's Hospital in need of transplantation.
METHODS
This case report describes the use of ABOi transplantation and describes the details of the total exchange transfusion prior to cardiopulmonary bypass.
RESULTS
After a successful intraoperative total exchange transfusion following the ABOi protocol, the patient's isohemagglutinin titers were 1 VC on postoperative day (POD) 1, and isohemagglutinin titer was <1 VC on POD 14. The patient had no signs of rejection and continued to recover.
CONCLUSIONS
Successful ABOi transplantation requires planning, an interdisciplinary approach, and clear closed-loop communication. Planning with the surgical and anesthesia teams is necessary for the hemodynamic stability of the patient during the total volume exchange as well as precautions put in place to ensure the blood products used in this procedure are correct. Planning with the lab and blood bank is also necessary to ensure they are prepared with enough blood products and can run isohemagglutinin titers.
Topics: Child; Humans; Hemagglutinins; Blood Group Incompatibility; Heart Transplantation; Cardiopulmonary Bypass; Exchange Transfusion, Whole Blood; Graft Rejection; ABO Blood-Group System
PubMed: 37378443
DOI: 10.1051/ject/2023009 -
AJOG Global Reports May 2023Hemolytic disease of the fetus and newborn (HDFN) is mediated by maternal alloantibodies, a consequence of immune sensitization during pregnancy with maternal-fetal...
BACKGROUND
Hemolytic disease of the fetus and newborn (HDFN) is mediated by maternal alloantibodies, a consequence of immune sensitization during pregnancy with maternal-fetal incompatibility with ABO, Rhesus factor (Rh), and/or other red blood cell antigens. RhD, Kell, and other non-ABO alloantibodies are the primary cause of moderate to severe HDFN, whereas ABO HDFN is typically mild. HDFN live birth prevalence owing to Rh alloimmunization among newborns in the United States was last estimated to be 106 per 100,000 births in 1986. HDFN live birth prevalence owing to all alloantibodies was estimated to be 817 to 840 per 100,000 in Europe. There is a need for updated prevalence estimates in the United States and a better understanding of disease demographics, severity, and treatments.
OBJECTIVE
This study aimed to estimate the live birth prevalence of HDFN and the proportion of severe cases of HDFN in the United States, to describe the associated risk factors, and to compare the clinical outcomes and treatments among healthy newborns, newborns with HDFN, and newborns who are sick without HDFN using a nationally representative hospital discharge database.
STUDY DESIGN
In this retrospective, observational cohort study, we used data from the 1996 to 2010 National Hospital Discharge Survey to identify live births, defined by inpatient visits with the newborn flag, with and without a diagnosis of HDFN across 200 to 500 sampled hospitals (≥6 beds) per year. Patient and hospital characteristics, alloimmunization status, disease severity, treatment, and clinical outcomes were evaluated. Frequencies and weighted percentages were calculated for all variables. Logistic regression was used to compare the characteristics between newborns with HDFN and other newborns using odds ratios.
RESULTS
Of 480,245 live births identified, 9810 HDFN cases were recorded. When weighted to the United States population, this corresponded to a live birth prevalence of 1695 per 100,000 live births. Compared with other newborns, newborns with HDFN were more likely to be female, Black, living in the South (vs the Midwest or West), and treated at larger (>100 beds) and government-owned hospitals. ABO and Rh alloimmunization accounted for 78.1% and 4.3% of newborns with HDFN, respectively, whereas HDFN caused by other antigens, such as Kell and Duffy, accounted for 17.6% of the cases. Among newborns with HDFN, 22% received phototherapy, 1% received simple transfusions, and 0.5% received exchange transfusions or intravenous immunoglobulin. Newborns affected by HDFN caused by Rh alloimmunization were more likely to require medical interventions, including simple or exchange transfusions, and more likely to be delivered by cesarean delivery. Overall, HDFN was associated with a longer hospital length of stay in the neonatal intensive care unit when compared with healthy and other sick newborns, a higher rate of cesarean delivery, and a higher rate of nonroutine discharge than healthy newborns.
CONCLUSION
Overall, the live birth prevalence of HDFN was higher than those previously reported, whereas Rh-induced HDFN live birth prevalence was similar to those previously reported. HDFN live birth prevalence owing to Rh alloimmunization decreased over time, likely because of continued Rh immune globulin prophylaxis. Treatment patterns for newborns with HDFN and the comparative clinical outcomes when compared with healthy newborns confirm the continued clinical needs of this population.
PubMed: 37229151
DOI: 10.1016/j.xagr.2023.100203 -
International Journal of Neonatal... Apr 2023Anti-Kell alloimmunisation is a potentially severe minor blood group type incompatibility, not only as a cause of haemolytic disease of the foetus and newborn, but also...
Anti-Kell alloimmunisation is a potentially severe minor blood group type incompatibility, not only as a cause of haemolytic disease of the foetus and newborn, but also due to the destruction of red blood cells (RBC) and mature form in the bone marrow with the subsequent hyporegenerative anaemia. In severe cases and when the foetus shows signs of anaemia, an intrauterine transfusion (IUT) may be necessary. When repeated, this treatment can suppress erythropoiesis and worsen the anaemia. We report the case of a newborn who required four IUTs plus an additional RBC transfusion at one month of life due to late onset anaemia. The identification of an adult haemoglobin profile with a complete absence of foetal haemoglobin in the patient's newborn screening samples at 2 and 10 days of life warned us of a possible late anaemia. The newborn was successfully treated with transfusion, oral supplements and subcutaneous erythropoietin. A blood sample taken at 4 months of life showed the expected haemoglobin profile for that age with a foetal haemoglobin of 17.7%. This case illustrates the importance of a close follow-up of these patients, as well as the usefulness of the haemoglobin profile screening as a tool for anaemia assessment.
PubMed: 37218889
DOI: 10.3390/ijns9020024 -
Asian Journal of Transfusion Science 2023The P blood group system was introduced in 1927 by Landsteiner and Levine. About 75% of the population possesses P1 phenotype. P2 simply implies P1 negative and there is...
The P blood group system was introduced in 1927 by Landsteiner and Levine. About 75% of the population possesses P1 phenotype. P2 simply implies P1 negative and there is no P2 antigen. Individuals with P2 may have anti-P1 antibodies in there serum are cold-reacting antibodies which are clinically insignificant and occasionally active at 20°C or higher temperatures. However, in certain cases, anti-P1 is clinically significant and may cause acute intravascular hemolytic transfusion reactions. Our case report confirms the complexity and difficulty in the diagnosis of anti-P1. In India, very few cases are reported regarding clinical significant anti-P1. Here, we report a case of IgM type of antibody anti-P1 which was reactive at 37°C and AHG phase in a 66-year-old female planned for Whipple's surgery, who had grouping discrepancies in reverse typing and incompatibility during routine crossmatch.
PubMed: 37188025
DOI: 10.4103/ajts.ajts_165_21 -
Asian Journal of Transfusion Science 2023ABO antibodies are naturally occurring antibodies. The ABO antibodies found in the Group O individuals include anti-A and anti-B. In Group O individuals, it tends to be...
ABO antibodies are naturally occurring antibodies. The ABO antibodies found in the Group O individuals include anti-A and anti-B. In Group O individuals, it tends to be predominantly immunoglobulins G (IgG), although immunoglobulins M and IgA components are also present. Infants of Group O mothers are at higher risk for hemolytic disease of the fetus and new-born than those born to mothers with Group A or B because IgG readily cross the placenta. At the same time, abnormal high concentration of ABO antibody in mother can lead to destruction of platelets in neonates and leads to development of neonatal alloimmune thrombocytopenia as human platelets carry detectable quantities of A and B blood group antigens on their surface. Proper and early diagnosis combined with treatment with intravenous immunoglobulins or transfusion with compatible platelets, may be from mother, can save the neonate from bleeding episodes.
PubMed: 37188011
DOI: 10.4103/ajts.ajts_194_20