Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 134
Filtrar
1.
Br J Haematol ; 2024 Aug 08.
Artículo en Inglés | MEDLINE | ID: mdl-39118348

RESUMEN

The use of uncrewed aerial vehicles (drones) has increased over the last decade. However, their application in healthcare has not been fully examined, in part, due to regulations preventing flight beyond the visual line of sight. This prospective randomised controlled laboratory study aimed to determine whether the in vitro quality of packed red blood cell components is maintained when transported by drone, beyond visual line of sight. Ten identical pairs of packed red blood cell units were randomly allocated to transport by drone or by ground vehicle (1:1, allocation concealment) 68 km between two hospitals in Northumbria, UK. Markers of blood component quality were compared at 8, 14, 28 and 35 days following blood unit manufacture. There was no statistical difference in haemolysis, potassium concentration, total haemoglobin, glucose and lactate, haematocrit and mean cell volume, between the two groups, up to the date of unit expiry. The temperature of the packed red blood cell units did not deviate outside the recommended 2-10°C for transportation, regardless of the allocated group. Blood component transport was faster by drone, but did not reach statistical significance. This study demonstrates the feasibility and safety of flying blood components by drone between hospitals in the United Kingdom.

2.
Br J Haematol ; 2024 Aug 20.
Artículo en Inglés | MEDLINE | ID: mdl-39162344

RESUMEN

ABO blood group discrepancies in healthy individuals were caused by body-wide chimerism and mosaicism. They can be evaluated with new diagnostic options for disease-related cell clones that are typically associated with mosaicism. The observations raise the attention for sporadic mixed-field observations of any blood group antigen. Commentary on: Dauber et al. Body-wide chimerism and mosaicism are predominant causes of naturally occurring ABO discrepancies. Br J Haematol 2024 (Online ahead of print). doi:10.1111/bjh.19618.

3.
Br J Haematol ; 205(2): 613-623, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39118415

RESUMEN

Pyruvate kinase (PK) deficiency, a rare, congenital haemolytic anaemia caused by mutations in the PKLR gene, is associated with many clinical manifestations, but the full disease burden has yet to be characterised. The Peak Registry (NCT03481738) is an observational, longitudinal registry of adult and paediatric patients with PK deficiency. Here, we described comorbidities and complications in these patients by age at most recent visit and PKLR genotype. As of 13 May 2022, 241 patients were included in the analysis. In total, 48.3% had undergone splenectomy and 50.5% had received chelation therapy. History of iron overload (before enrolment/during follow-up) was common (52.5%), even in never-transfused patients (20.7%). Neonatal complications and symptoms included jaundice, splenomegaly and hepatomegaly, with treatment interventions required in 41.5%. Among adults, osteopenia/osteoporosis occurred in 19.0% and pulmonary hypertension in 6.7%, with median onset ages of 37, 33 and 22 years, respectively. Biliary events and bone health problems were common across PKLR genotypes. Among 11 patients who had thromboembolic events, eight had undergone prior splenectomy. Patients with PK deficiency may have many complications, which can occur early in and throughout life. Awareness of their high disease burden may help clinicians better provide appropriate monitoring and management of these patients.


Asunto(s)
Anemia Hemolítica Congénita no Esferocítica , Piruvato Quinasa , Errores Innatos del Metabolismo del Piruvato , Sistema de Registros , Humanos , Piruvato Quinasa/deficiencia , Piruvato Quinasa/genética , Masculino , Femenino , Adulto , Niño , Anemia Hemolítica Congénita no Esferocítica/genética , Anemia Hemolítica Congénita no Esferocítica/epidemiología , Errores Innatos del Metabolismo del Piruvato/genética , Errores Innatos del Metabolismo del Piruvato/epidemiología , Adolescente , Preescolar , Lactante , Comorbilidad , Persona de Mediana Edad , Esplenectomía , Adulto Joven , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/genética , Hipertensión Pulmonar/epidemiología , Sobrecarga de Hierro/etiología , Sobrecarga de Hierro/epidemiología , Enfermedades Óseas Metabólicas/etiología , Enfermedades Óseas Metabólicas/epidemiología , Recién Nacido
4.
Blood Cells Mol Dis ; 104: 102801, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-37951089

RESUMEN

Several syndromes affecting the red cell that mimic those induced by germline mutations may result from a somatic mutation that accompanies a myeloid malignancy. These syndromes are most notable in cases of myelodysplastic syndrome, but they are not limited to any one category of myeloid neoplasm. Their occurrence in males exceed the male predominance that is evident in myeloid neoplasms. The syndromes include disorders of globin chain synthesis (α- and ß-thalassemia), heme synthesis (erythropoietic porphyria and erythropoietic uroporphyria), red cell membrane structure (elliptocytosis and spherocytosis), red cell enzyme activity (pyruvate kinase deficiency, glucose-6-phosphate dehydrogenase deficiency) and lowered expression of red cell ABO blood group antigens. This historical review describes the path to uncovering these acquired syndromes and their causal somatic mutations, where known. These syndromes often go unrecognized because of the dominant concern of the primary neoplasm. They may add to the healthcare needs of the patient.


Asunto(s)
Anemia Hemolítica Congénita no Esferocítica , Síndromes Mielodisplásicos , Neoplasias , Humanos , Masculino , Femenino , Hematopoyesis Clonal , Eritrocitos/patología , Síndromes Mielodisplásicos/patología , Mutación , Hematopoyesis
5.
Transfusion ; 64(1): 141-149, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-38059437

RESUMEN

BACKGROUND: Each unit of red blood cells (RBCs) produced represents a significant cost to the healthcare system. Unnecessary blood wastage should be minimized. In clinical settings, alterations to blood component bags after issue from the protected setting of the blood bank include pen markings, and those that are exposed to an infectious environment require surface disinfecting. These units may be discarded due to unclear effects on RBC quality. In this study, we investigate whether pen markings or surface disinfection negatively affects the quality of packed RBCs and whether pen ink diffuses through the blood bag. STUDY DESIGN AND METHODS: RBC bags were marked with pens (water, oil, or alcohol-based) or subjected to surface disinfection (ethanol, hydrogen peroxide [Preempt wipes], or benzalkonium chloride-based wipes [CaviWipes]) and sampled 24 h after applying the treatment and at day 42 post collection (n = 3 for each condition). The samples were analyzed for RBC in vitro quality markers. The presence of any ink in the RBC bags was investigated using mass spectrometry (n = 2). RESULTS: Data from 24 h and day 42 time points indicated no differences in RBC count, mean corpuscular volume, morphology, deformability, potassium content, or hemolysis for either pen markings or disinfectants when compared with their untreated controls (p > .05). No trace of ink was detected inside the bag. CONCLUSION: RBC units marked with ballpoint, gel, or Sharpie pens do not suffer a loss of in vitro quality, nor do RBC units which have been surface disinfected with 70% ethanol, Preempt wipes or CaviWipes.


Asunto(s)
Desinfectantes , Humanos , Desinfectantes/farmacología , Tinta , Conservación de la Sangre/métodos , Eritrocitos , Cloruro de Polivinilo/química , Cloruro de Polivinilo/farmacología , Etanol/farmacología , Compuestos Orgánicos
6.
Transfusion ; 64(6): 998-1007, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38689458

RESUMEN

BACKGROUND: Current hemovigilance methods generally rely on survey data or administrative claims data utilizing billing and revenue codes, each of which has limitations. We used electronic health records (EHR) linked to blood bank data to comprehensively characterize red blood cell (RBC) utilization patterns and trends in three healthcare systems participating in the U.S. Food and Drug Administration Center for Biologics Evaluation and Research Biologics Effectiveness and Safety (BEST) initiative. METHODS: We used Information Standard for Blood and Transplant (ISBT) 128 codes linked to EHR from three healthcare systems data sources to identify and quantify RBC-transfused individuals, RBC transfusion episodes, transfused RBC units, and processing methods per year during 2012-2018. RESULTS: There were 577,822 RBC units transfused among 112,705 patients comprising 345,373 transfusion episodes between 2012 and 2018. Utilization in terms of RBC units and patients increased slightly in one and decreased slightly in the other two healthcare facilities. About 90% of RBC-transfused patients had 1 (~46%) or 2-5 (~42%)transfusion episodes in 2018. Among the small proportion of patients with ≥12 transfusion episodes per year, approximately 60% of episodes included only one RBC unit. All facilities used leukocyte-reduced RBCs during the study period whereas irradiated RBC utilization patterns differed across facilities. DISCUSSION: ISBT 128 codes and EHRs were used to observe patterns of RBC transfusion and modification methods at the unit level and patient level in three healthcare systems participating in the BEST initiative. This study shows that the ISBT 128 coding system in an EHR environment provides a feasible source for hemovigilance activities.


Asunto(s)
Registros Electrónicos de Salud , Transfusión de Eritrocitos , Humanos , Femenino , Masculino , Persona de Mediana Edad , Adulto , Estados Unidos , Eritrocitos , Anciano , Productos Biológicos/uso terapéutico , Bancos de Sangre/normas , Bancos de Sangre/estadística & datos numéricos , Adolescente
7.
Transfusion ; 64(7): 1296-1305, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38817044

RESUMEN

BACKGROUND: CD59 deficiency due to rare germline variants in the CD59 gene causes disabilities, ischemic strokes, neuropathy, and hemolysis. CD59 deficiency due to common somatic variants in the PIG-A gene in hematopoietic stem cells causes paroxysmal nocturnal hemoglobinuria. The ISBT database lists one nonsense and three missense germline variants that are associated with the CD59-null phenotype. To analyze the genetic diversity of the CD59 gene, we determined long-range CD59 haplotypes among individuals from different ethnicities. METHODS: We determined a 22.7 kb genomic fragment of the CD59 gene in 113 individuals using next-generation sequencing (NGS), which covered the whole NM_203330.2 mRNA transcript of 7796 base pairs. Samples came from an FDA reference repository and our Ethiopia study cohorts. The raw genotype data were computationally phased into individual haplotype sequences. RESULTS: Nucleotide sequencing of the CD59 gene of 226 chromosomes identified 216 positions with single nucleotide variants. Only three haplotypes were observed in homozygous form, which allowed us to assign them unambiguously as experimentally verified CD59 haplotypes. They were also the most frequent haplotypes among both cohorts. An additional 140 haplotypes were imputed computationally. DISCUSSION: We provided a large set of haplotypes and proposed three verified long-range CD59 reference sequences, based on a population approach, using a generalizable rationale for our choice. Correct long-range haplotypes are useful as template sequences for allele calling in high-throughput NGS and precision medicine approaches, thus enhancing the reliability of clinical diagnostics. Long-range haplotypes can also be used to evaluate the influence of genetic variation on the risk of transfusion reactions or diseases.


Asunto(s)
Antígenos CD59 , Haplotipos , Humanos , Antígenos CD59/genética , Secuenciación de Nucleótidos de Alto Rendimiento/métodos , Etnicidad/genética , Masculino , Femenino , Polimorfismo de Nucleótido Simple , Anemia Hemolítica , Hemoglobinuria
8.
Transfusion ; 64(6): 1097-1108, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38716879

RESUMEN

BACKGROUND: N-(-9 acridinyl)-b-alanine hydrochloride (S-300) is the main byproduct of red blood cell (RBC) amustaline/glutathione(GSH) pathogen reduction, currently undergoing phase III US clinical trials following successful European studies(1-3). Phosphatidylinositol glycan, class A (Pig-a) X-linked gene mutagenesis is a validated mammalian in vivo mutation assay for genotoxicity, assessed as clonal loss of glycosylphosphatidylinositol-linked CD59 cell-surface molecules on reticulocytes (RETs) and RBCs. METHODS: Male Sprague-Dawley rats received continuous infusion of S-300 up to the maximum feasible dose (240 mg/kg/day-limited by solubility and volume) for 28 days. Positive controls received a known mutagen by oral gavage on Days 1-3. Plasma levels of S-300 were assessed by HPLC before, during and after infusion. CD59-negative RBCs and RETs were enumerated in pre-dose and Day 28 samples, using a flow cytometric method. Outcome was evaluated by predetermined criteria using concurrent and historical controls. Toxicity was assessed by laboratory measures and necropsy. RESULTS: S-300 reached maximum, dose-dependent levels (3-15 µmol/L) within 2-8 h that were sustained for 672 h and undetectable 2 h after infusion. Circulating RET levels indicated a lack of hematopoietic toxicity. Necropsy revealed minimal-mild observations related to poor S-300 solubility at high concentrations. Pig-a assessment met the preset acceptability criteria and revealed no increase in mutant RBCs or RETs. CONCLUSIONS: Maximum feasible S-300 exposure of rats by continuous infusion for 28 days was not genotoxic as assessed by an Organization for Economic Cooperation and Development-compliant, mammalian, in vivo Pig-a gene mutation assay that meets the requirements of International Conference on Harmonization (ICH) S2(R1) and FDA guidances on genotoxicity testing.


Asunto(s)
Pruebas de Mutagenicidad , Ratas Sprague-Dawley , Animales , Masculino , Ratas , Pruebas de Mutagenicidad/métodos , Antígenos CD59/genética , Reticulocitos/efectos de los fármacos , Eritrocitos/efectos de los fármacos , Eritrocitos/metabolismo , Proteínas de la Membrana/genética , Mutagénesis/efectos de los fármacos , Mutágenos/toxicidad
9.
Transfusion ; 2024 Aug 02.
Artículo en Inglés | MEDLINE | ID: mdl-39095932

RESUMEN

BACKGROUND: The cellular and molecular changes during red blood cell (RBC) storage that affect posttransfusion recovery (PTR) remain incompletely understood. We have previously reported that RBCs of different storage biology cross-regulate each other when stored together (co-storage cross-regulation [CSCR]). However, the mechanism of CSCR is unclear. In the current study, we tested the hypothesis that CSCR involves acquisition of molecular signatures associated with PTR. STUDY DESIGN AND METHODS: The whole blood compartment of either B6 or FVB mice was biotinylated in vivo prior to blood collection and storage. Bio-B6 or Bio.FVB were stored with RBCs from B6 mice transgenic for green florescent protein (GFP) (B6.GFP). After storage, avidin-magnetic beads were used to simultaneous purify Bio-RBCs (positive selection) and B6.GFPs (negative selection). Isolated populations were analyzed by transfusion to establish PTR, and subjected to metabolomic and proteomic analysis. RESULTS: B6 RBCs acquired molecular signatures associated with stored FVB RBCs at both the metabolomic and proteomic level including metabolites associated with energy metabolism, oxidative stress regulation, and oxidative damage. Mitochondrial signatures were also acquired by B6 RBCs. Protein signatures acquired by B6 RBCs include proteins associated with vesiculation. CONCLUSION: The data presented herein demonstrate the appearance of multiple molecular changes from poor-storing RBCs in good-storing RBCs during co-storage. Whether this is a result of damage causing intrinsic molecular changes in B6 RBCs or if molecules of FVB RBC origin are transferred to B6 RBCs remains unclear. These studies broaden our mechanistic understanding of RBC storage (in particular) and potentially RBC biology (in general).

10.
Transfusion ; 64(2): 223-235, 2024 02.
Artículo en Inglés | MEDLINE | ID: mdl-38323704

RESUMEN

BACKGROUND: The optimal hemoglobin (Hb) threshold for red blood cell transfusions in adult patients with myelodysplastic syndromes (MDS) has not been defined. STUDY DESIGN AND METHODS: We conducted a pilot randomized multi-center study of two transfusion algorithms (liberal, to maintain Hb 110-120 g/L, transfuse 2 units if Hb < 105 g/L and 1 unit if Hb 105-110 g/L vs. restrictive, 85-105 g/L, transfuse 2 units when Hgb < 85 g/L). Primary objectives were 70% compliance in maintaining the q2 week hemoglobin within the targeted range and the achievement of a 15 g/L difference in pre-transfusion Hb. Secondary outcomes included measures of quality of life (QOL), iron studies and safety. RESULTS: Twenty-eight patients were randomized between February 2015-2020, 13 to the restrictive arm and 15 to the liberal arm in three tertiary care centers. The compliance was 66% and 45% and the mean pre-transfusion Hb thresholds were 86 (standard deviation [SD] 8) and 98 g/L (SD 10) in the restrictive and liberal arms, (mean difference 11.8 g/L, p < .0001), respectively. Patients in the liberal arm experienced a mean of 3.4 (SD 2.6) more transfusion visits and received a mean of 5.3 (SD 5.5) more units of blood during the 12-week study. Ferritin increased by 1043 (SD 1516) IU/L and 148 (SD 1319) IU/L in the liberal and restrictive arms, respectively. Selected QOL scores were superior pre-transfusion and more patients achieved clinically important improvements in the liberal arm compared with the restrictive arm for selected symptoms and function domains. CONCLUSION: The results establish that policies for transfusion support can be delivered in practice at multiple hospitals, but further research is required to understand the full clinical effects and safety of liberal transfusion policies in MDS outpatients.


Asunto(s)
Transfusión de Eritrocitos , Síndromes Mielodisplásicos , Adulto , Humanos , Transfusión de Eritrocitos/métodos , Calidad de Vida , Pacientes Ambulatorios , Proyectos Piloto , Síndromes Mielodisplásicos/terapia , Hemoglobinas/análisis
11.
Blood Cells Mol Dis ; 103: 102778, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37379758

RESUMEN

Nineteen reports of 41 cases of acquired red cell elliptocytosis associated with a chronic myeloid neoplasm are described. Although the majority of cases have an abnormality of the long arm of chromosome 20, del(q20), several cases do not. Moreover, in one case a specific qualitative abnormality of red cell protein band 4.1(4.1R) was reported; however, several subsequent cases could find no abnormality of a red cell membrane protein or found a different abnormality, usually quantitative. Thus, this striking red cell phenotypic feature, acquired elliptocytosis, seen in myelodysplastic syndrome and other chronic myeloproliferative diseases, closely simulating the red cell phenotype of hereditary elliptocytosis, has an unexplained genetic basis, presumably as the result of an acquired mutation(s) in some chronic myeloid neoplasms.


Asunto(s)
Eliptocitosis Hereditaria , Trastornos Mieloproliferativos , Neoplasias , Humanos , Eliptocitosis Hereditaria/complicaciones , Eliptocitosis Hereditaria/genética , Proteínas de la Membrana/genética , Neoplasias/metabolismo , Eritrocitos/metabolismo , Membrana Eritrocítica/metabolismo , Trastornos Mieloproliferativos/complicaciones , Trastornos Mieloproliferativos/genética , Trastornos Mieloproliferativos/metabolismo , Proteínas del Citoesqueleto/genética
12.
Transfusion ; 63(10): 1841-1848, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37698202

RESUMEN

BACKGROUND: We recently introduced a policy to use O positive red cells in emergency transfusions for males >16 years of age and females >50 years of age. Here, we investigate changes in emergency transfusion practice and rates of red cell alloimmunization with the use of O positive blood for emergency transfusion. STUDY DESIGN AND METHODS: State-wide retrospective review of emergency transfusions between June 2020 and June 2021. The laboratory information system and patient medical records were used to collect demographic details, indications for transfusion, usage of O positive and O negative blood and rates of alloimmunization. RESULTS: There were 2354 red cell units transfused to 1013 patients (male = 59%, average age = 53 years) during the 12-month period. O positive units accounted for 46.9% (1103 units) of emergency transfusions. However, 726 (30.8%) O negative units were transfused to patients without a mandatory indication for O negative blood. Twenty-eight patients (2.9%) had a red cell alloantibody prior to transfusion including anti-E (n = 10), anti-D (n = 4), and anti-K (n = 4). One patient with prior anti-D had mild delayed hemolysis. There were 19 patients (4.3%, median follow-up 22 days) who developed a red cell alloantibody after emergency transfusion and include anti-E (n = 10), anti-D (n = 7), and anti-C (n = 5). DISCUSSION: The use of O positive blood for emergency transfusion has saved 1103 O negative red cell units with no detriment to patient outcome. There remains potential to optimize use of O positive blood in emergency transfusion and to understand red cell alloimmunization rates in a prospective fashion.

13.
Transfusion ; 63(9): 1685-1691, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37587736

RESUMEN

BACKGROUND: Intravenous (IV) iron carries risks of mild, self-limiting, tryptase-negative Fishbane and complement activation-related pseudo-allergy reactions, with rare reports of anaphylaxis. Historically, high-molecular-weight iron dextran (HMWID) was associated with a higher incidence of anaphylaxis and empiric premedication with antihistamines/corticosteroids have been used to mitigate this risk. HMWID is no longer available and the risk of hypersensitivity reactions with newer IV iron formulations is low. Therefore, the use of routine prophylactic premedication in all patients is not justified but should be considered in high-risk patients. STUDY DESIGN AND METHODS: Our primary aim was to reduce inappropriate premedication before IV iron administration by 50% so that our institution's hematology providers only prescribe premedications to patients at high risk of having a severe reaction. Interventions included a multidisciplinary education initiative to highlight current evidence against universal administration of premedications and revision of the IV iron informed consent form and electronic order set. RESULTS: We measured the success of our intervention by comparing data collected during a 6-month pre-intervention period (837 infusions) to a 6-month post-intervention period (947 infusions). Inappropriate administration of premedications decreased from 79% in the pre-intervention period compared to 65% in the post-intervention period. We found no significant difference in the number of Fishbane reactions, severe reactions, and emergency room admissions, despite this reduction in premedication use. DISCUSSION: Although we did not reach our goal of a 50% reduction in inappropriate premedication use, opportunities for process improvements were uncovered and are being explored in the next cycle of this quality improvement project.


Asunto(s)
Anafilaxia , Humanos , Anafilaxia/prevención & control , Mejoramiento de la Calidad , Hierro/uso terapéutico , Complejo Hierro-Dextran , Administración Intravenosa
14.
Transfusion ; 63 Suppl 1: S33-S36, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36748667

RESUMEN

BACKGROUND: Fat embolism syndrome (FES) is a rare complication, which was reported mostly with milder forms of heterozygous sickle cell disease (SCD). It may present in a catastrophic way with multi-organ failure, particularly involving the pulmonary and neurological systems. Diagnosis is often missed or delayed; and the standard recommended treatment is red cell exchange (RCE) transfusion, which has sub-optimal results, such as debilitating long-term neurological complications. Recently, few reports suggested that the addition of Therapeutic Plasma Exchange (TPE) might further improve the outcome. CASE DESCRIPTION: A 23-year-old woman with homozygote SCD was admitted with bony pains and vaso-occlusive crises. However, her course evolved to respiratory failure requiring mechanical ventilation, decreased level of consciousness, skin rash, severe anemia and thrombocytopenia and a picture consistent with thrombotic microangiopathy. MRI of the brain showed scattered multi-focal ischemic foci and cytotoxic edema. The patient received RCE on the third day after admission without improvement. On the seventh day, TPE was instituted (2 L/day of fresh frozen plasma for 5 days), following which she regained her consciousness and showed an improvement in her laboratory abnormalities. On follow up, she had gradual full neurological recovery and resolution of the MRI findings within a few months. CONCLUSION: FES remains a diagnostic and therapeutic challenge, with significant morbidity and mortality. Success in the management of this reported case with the addition of TPE to RCE supports the notion that TPE may be a potentially helpful modality that deserves further research.


Asunto(s)
Anemia de Células Falciformes , Embolia Grasa , Humanos , Femenino , Adulto Joven , Adulto , Intercambio Plasmático , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/terapia , Insuficiencia Multiorgánica/terapia , Plasma , Embolia Grasa/terapia , Embolia Grasa/complicaciones
15.
Transfusion ; 63(10): 1969-1977, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37632701

RESUMEN

BACKGROUND: Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia (AIHA), mainly affecting children. The diagnosis and management are challenging due to similarities to other causes for AIHA and limited availability to Donath-Landsteiner (DL) testing. STUDY DESIGN AND METHODS: In this single-center retrospective study, we aimed to characterize the clinical presentation and outcomes of PCH patients, defined as having positive Donath-Landsteiner antibodies, compared to a cohort of AIHA patients. RESULTS: DL-positive patients were observed to have higher lactate dehydrogenase levels and lower reticulocyte counts compared to DL-negative patients, although this was not statistically significant. We also observed that using steroids in DL-positive patients did not significantly impact their recovery. DISCUSSION: Our findings support the limited published data on PCH patients and further prompt larger multicenter studies to further characterize these patients so that they are more readily identified, especially in centers where DL antibody testing is not readily available.

16.
Transfusion ; 63(4): 867-871, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36727661

RESUMEN

BACKGROUND: Cisplatin-associated hemolysis is a rare but important adverse effect. Nonimmunological protein adsorption (NIPA) due to erythrocyte membrane modification has been reported as the leading cause of cisplatin-associated hemolysis. However, limited data exist on cisplatin-associated immunological hemolysis because of a lack of an established diagnostic method. Here, we used flow cytometry (FCM) to diagnose a patient with cisplatin-associated immunological hemolysis. STUDY DESIGN AND METHODS: A 55-year-old woman with uterocervical cancer was treated with weekly cisplatin monotherapy (40 mg/m2 ). She had no previous transfusion and medication history, nor any significant family history. On the 26th day after cisplatin administration, severe hemolysis was noted. Her red blood cells (RBCs) and sera were evaluated by direct antiglobulin test (DAT) and indirect antiglobulin test (IAT), respectively. To explore immunological reactions for cisplatin-treated RBCs, we attempted FCM using cisplatin-treated and -untreated RBCs. After incubating conditioned RBCs with the patient's serum or healthy donor serum, we evaluated their fluorescent intensity by fluorescein isothiocyanate (FITC)-conjugated anti-human immunoglobulin (Ig) G antibodies. RESULTS: The patient's DAT was positive, and an IAT using her plasma was positive for cisplatin-treated RBCs. FCM using cisplatin-treated RBCs revealed that the patient's serum had higher FITC intensity than the donor's serum, indicating the existence of cisplatin-treated RBC-specific IgGs in patient's serum. CONCLUSION: Here, we report a rare case of a patient with hemolysis diagnosed using FCM to identify specific antibodies against cisplatin-treated RBCs. NIPA and immunological mechanisms may contribute to hemolysis onset during cisplatin treatment.


Asunto(s)
Anticuerpos , Cisplatino , Humanos , Femenino , Persona de Mediana Edad , Cisplatino/efectos adversos , Cisplatino/metabolismo , Fluoresceína-5-Isotiocianato/metabolismo , Citometría de Flujo , Anticuerpos/metabolismo , Eritrocitos/metabolismo , Hemólisis , Proteínas
17.
Transfusion ; 63(3): 610-618, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36744388

RESUMEN

BACKGROUND: An antibody directed against a high-prevalence red blood cell (RBC) antigen was detected in a 67-year-old female patient of North African ancestry with a history of a single pregnancy and blood transfusion. So far, the specificity of the proband's alloantibody remained unknown in our immunohematology reference laboratory. STUDY DESIGN AND METHODS: Whole-exome sequencing (WES) was performed on the proband's DNA. The reactivity to the SLC29A1-encoded ENT1 adenosine transporter was investigated by flow cytometry analyses of ENT1-expressing HEK293 cells, and RBCs from Augustine-typed individuals. Erythrocyte protein expression level, nucleoside-binding capacity, and molecular structure of the proband's ENT1 variant were further explored by western blot, flow cytometry, and molecular dynamics calculations, respectively. RESULTS: A missense variant was identified in the SLC29A1 gene, which encodes the Augustine blood group system. It arises from homozygosity for a rare c.242A > G missense mutation that results in a nonsynonymous p.Asn81Ser substitution within the large extracellular loop of ENT1. Flow cytometry analyses demonstrated that the proband's antibody was reactive against HEK-293 cells transfected with control but not proband's SLC29A1 cDNA. Consistent with this finding, proband's antibody was found to be reactive with At(a-) (AUG:-2), but not AUG:-1 (null phenotype) RBCs. Data from structural analysis further supported that the proband's p.Asn81Ser variation does not alter ENT1 binding of its specific inhibitor NBMPR. CONCLUSION: Our study provides evidence for a novel high-prevalence antigen, AUG4 (also called ATAM after the proband's name) in the Augustine blood group system, encoded by the rare SLC29A1 variant allele AUG*04 (c.242A > G, p.Asn81Ser).


Asunto(s)
Antígenos de Grupos Sanguíneos , Embarazo , Femenino , Humanos , Células HEK293 , Prevalencia , Antígenos de Grupos Sanguíneos/genética , Isoanticuerpos , Estructura Molecular
18.
Transfusion ; 63(5): 952-959, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-37005945

RESUMEN

BACKGROUND: Good models of chronic red blood cell (RBC) transfusion can improve care by accurately estimating time-dependent hemoglobin (Hb) levels and clinically relevant transfusion parameters. STUDY DESIGN AND METHODS: A previously derived mathematical model based on overall Hb mass balance (HMB model) with input parameters of transfused units, transfusion efficiency, RBC lifespan, endogenous Hb, and transfusion interval was applied to three separate clinical studies spanning six transfusion conditions across patient populations with myelodysplastic syndrome (MDS) or thalassemia. RESULTS: The HMB model accurately predicted mean pretransfusion Hb levels for each ensemble cohort of patients with thalassemia or MDS. Dynamic changes in Hb levels were modeled as a function of changes in key input parameters. Improving the 24-h post-transfusion RBC survival from 72% to 86% can be used to either (1) reduce RBC usage by 15%-20% through longer transfusion intervals or (2) increase pretransfusion [Hb] by 8%-11% while maintaining a constant transfusion interval. DISCUSSION: The endogenous Hb level is introduced in the HMB model which represents the patient's self-contribution to overall Hb levels via the autologous RBC lifecycle and was estimated to be 5.0 g/dL for patients with MDS or thalassemia. Transfusion therapy approaches and complementary therapeutics can target multiple, unique model inputs while monitoring net, overall impact on transfusion efficacy. Applying the HMB model to fit individual patient Hb fluctuations will be explored in the future.


Asunto(s)
Síndromes Mielodisplásicos , Talasemia , Humanos , Hemoglobinas , Eritrocitos , Transfusión Sanguínea , Transfusión de Eritrocitos , Síndromes Mielodisplásicos/terapia
19.
Transfusion ; 62(8): 1519-1526, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35657149

RESUMEN

BACKGROUND: Guidelines recommend transfusion of red blood cells (RBC's) when a hospitalized patient's hemoglobin (Hb) drops below a restrictive transfusion threshold, either at 7 or 8 g. Hospitals have implemented transfusion policies to encourage compliance with guidelines and reduce variation in transfusion practice. However, variation in transfusion practice remains. The purpose of this study was to examine whether there is variation in the receipt of transfusion by patient race. METHODS: Hospitalized general medicine patients with anemia (Hb < 10 g/dL) were eligible. Chi-squared tests were used to compare the percent of patients receiving a transfusion by race overall and within strata of their nadir Hb. Linear regression was used to test the association between a patient's race, their nadir Hb, receipt of an RBC transfusion, and the number of units transfused. RESULTS: Four thousand nine hundred and fifty-one patients consented, including 1363 (28%) who received a transfusion. 71% of patients were African American, 25% were White, and 4% were Other Race. Overall African Americans were less likely to be transfused compared to Whites (25% vs. 30%, p < .01), and within Hb strata below a Nadir Hb of 9 g/dL (Hb 8.0-8.9 g/dL 1% vs. 7%, p < .01; 7.0-7.9 g/dL 15% vs. 28%, p < .01; <7 g/dL 80% vs. 86%, p < .01). African Americans also received fewer units of RBC's (ß = -.17, p < .01) overall and at lower Hb levels (ß = .14, p < .01) compared to Whites. DISCUSSION: The Hb level at which patients are transfused at and the total number of RBC units received during hospitalization differ by patient race.


Asunto(s)
Anemia , Transfusión de Eritrocitos , Anemia/terapia , Transfusión Sanguínea , Hemoglobinas/análisis , Humanos , Factores Raciales
20.
Vox Sang ; 117(5): 701-707, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-35018634

RESUMEN

BACKGROUND AND OBJECTIVES: D-negative red cells are transfused to D-negative females of childbearing potential (CBP) to prevent haemolytic disease of the foetus and newborn (HDFN). Transfusion of low-titre group O whole blood (LTOWB) prehospital is gaining interest, to potentially improve clinical outcomes and for logistical benefits compared to standard of care. Enhanced donor selection requirements and reduced shelf-life of LTOWB compared to red cells makes the provision of this product challenging. MATERIALS AND METHODS: A universal policy change to the use of D-positive LTOWB across England was modelled in terms of risk of three specific harms occurring: risk of haemolytic transfusion reaction now or in the future, and the risk of HDFN in future pregnancies for all recipients or D-negative females of CBP. RESULTS: The risk of any of the three harms occurring for all recipients was 1:14 × 103 transfusions (credibility interval [CI] 56 × 102 -42 × 103 ) while for females of CBP it was 1:520 transfusions (CI 250-1700). The latter was dominated by HDFN risk, which would be expected to occur once every 5.7 years (CI 2.6-22.5). We estimated that a survival benefit of ≥1% using LTOWB would result in more life-years gained than lost if D-positive units were transfused exclusively. These risks would be lower, if D-positive blood were only transfused when D-negative units are unavailable. CONCLUSION: These data suggest that the risk of transfusing RhD-positive blood is low in the prehospital setting and must be balanced against its potential benefits.


Asunto(s)
Servicios Médicos de Urgencia , Eritroblastosis Fetal , Reacción a la Transfusión , Sistema del Grupo Sanguíneo ABO , Transfusión Sanguínea , Inglaterra , Femenino , Hemorragia/etiología , Hemorragia/terapia , Humanos , Recién Nacido , Embarazo , Resucitación , Reacción a la Transfusión/prevención & control
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA