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BACKGROUND: Regulatory aspects of transfusion medicine add complexity in blinded transfusion trials when considering various electronic record keeping software and blood administration processes. The aim of this study is to explore strategies when blinding transfusion components and products in paper and electronic medical records. METHODS: Surveys were collected and interviews were conducted for 18 sites across various jurisdictions in North America to determine solutions applied in previous transfusion randomized control trials. RESULTS: Sixteen responses were collected of which 11 had previously participated in a transfusion randomized control trial. Various solutions were reported which were specific to the laboratory information system (LIS) and electronic medical record (EMR) combinations although solutions could be grouped into four categories which included the creation of a study product code in the LIS, preventing the transmission of data from the LIS to the EMR, utilizing specialized stickers and labels to conceal product containers and documents in the paper records, and modified bedside procedures and documentation. DISCUSSION: LIS and EMR combinations varied across sites, so it was not possible to determine combination-specific solutions. The study was able to highlight solutions that may be emphasized in future iterations of LIS and EMR software as well as procedural changes that may minimize the risk of unblinding.
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Transfusión Sanguínea , Registros Electrónicos de Salud , Humanos , Transfusión de Componentes Sanguíneos , América del Norte , Proyectos de Investigación , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
INTRODUCTION: Individuals with sickle cell disease (SCD) and central venous catheters (CVC) are at high risk for venous thromboembolism (VTE). Minimal data exist regarding the use of anticoagulation as thromboprophylaxis of VTE in this demographic, and as a result, clinical equipoise exists. Prophylactic dose rivaroxaban, a direct oral anticoagulant, is efficacious and safe as thromboprophylaxis in other demographics, and may be an optimal agent in SCD with CVC. Prior to conducting a full clinical trial to assess rivaroxaban as thromboprophylaxis in SCD with CVC, a pilot study is needed to gauge its feasibility. METHODS AND ANALYSIS: THromboprophylaxis In Sickle Cell Disease pilot trial is an investigator-initiated, multicentre, double-blinded, randomised controlled trial (RCT) assessing if it is feasible and safe to conduct an adequately powered RCT comparing rivaroxaban to matching placebo as thromboprophylaxis in those with SCD and CVC. Fifty adult patients with SCD and CVC will be randomised to receive either rivaroxaban 10 mg daily or matching placebo for the duration of the CVC in situ for up to 1 year. After randomisation, follow-up visits will occur every 3 months. The primary outcomes pertain to the feasibility of a full trial and include numbers of eligible and recruited participants. Exploratory outcomes include overall incidence of VTE and bleeding complications, as well as quality of life. If the full trial is feasible, blinding will be maintained and patients in the pilot study will be included in the full trial. ETHICS AND DISSEMINATION: The trial was initially approved by the University Health Network Research Ethics Board (REB) in Toronto, Canada. All sites will obtain approval from their respective REB prior to commencement of study activities. Study results will be disseminated through presentations at medical conferences and peer-reviewed publications. TRIAL REGISTRATION NUMBER: NCT05033314.
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Anemia de Células Falciformes , Catéteres Venosos Centrales , Tromboembolia Venosa , Adulto , Humanos , Proyectos Piloto , Rivaroxabán/uso terapéutico , Catéteres Venosos Centrales/efectos adversos , Tromboembolia Venosa/etiología , Tromboembolia Venosa/prevención & control , Tromboembolia Venosa/tratamiento farmacológico , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/tratamiento farmacológico , Anticoagulantes/uso terapéutico , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
The International Society of Thrombosis and Hemostasis (ISTH) provides objective disseminated intravascular coagulation (DIC) measurement through diagnostic criteria validated in adults. The applicability of these criteria in pediatric and neonatal DIC is controversial and unvalidated. Primary objective: to evaluate current practice in pediatric and neonatal DIC management among different specialties. Secondary objective: to understand the potential impact of developmental hemostasis on DIC laboratory evaluation. We performed a multicenter survey between January and September 2016. The questionnaire was distributed internationally through professional societies. In all, 211 responses were received, of which 160 were full responses and 51 were partial. Overall, 85% of respondents practiced in tertiary academic centers; 70% practiced in pediatric-only hospitals. The majority of respondents (42%) used their personal clinical experience in the management of DIC. Sixty percent of respondents treated DIC until the resolution of both clinical and laboratory parameters. Laboratory investigations were monitored in the majority of DIC cases without thrombosis or bleeding (80%); age-specific reference ranges for tests were lacking in 20% of pediatric-only hospitals and 35% of combined pediatric/adult hospitals. Adherence to standardized DIC guidelines was poor but varied by geographical location. This survey reveals variable practices among pediatricians in the management of DIC. Further studies are needed to validate the DIC diagnostic criteria in children.
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Coagulación Intravascular Diseminada , Trombosis , Adulto , Recién Nacido , Humanos , Niño , Coagulación Intravascular Diseminada/diagnóstico , Coagulación Intravascular Diseminada/etiología , Coagulación Intravascular Diseminada/terapia , Salud del Lactante , Hemostasis , Trombosis/terapia , Encuestas y CuestionariosRESUMEN
AIMS AND OBJECTIVES: The aim of this study was to investigate nurse perspectives on transfusion-related adverse reaction reporting practices. BACKGROUND: Transfusion-related adverse reaction reporting is an essential component of hemovigilance in Canada, but reporting rates vary and under-reporting of minor transfusion-related adverse reactions exists. To our knowledge, this is the first report of nursing transfusion-related adverse reaction reporting attitudes. DESIGN: This qualitative descriptive study explored the nursing practices and perspectives of transfusion-related adverse reaction reporting by conducting one-on-one interviews with nurses (n = 25) working in adult oncology inpatient and outpatient units. METHODS: Data were thematically analysed; data collection ended when saturation was reached. The COREQ checklist was used to guide this study. RESULTS: The study revealed that the nursing practices of transfusion-related adverse reaction reporting are not standardised to meet the institutional reporting guidelines. Under-reporting of febrile reactions exists at this institution. Major concepts uncovered included the factors impacting nurses' transfusion-related reporting practices, as well as barriers and facilitators to transfusion reporting. CONCLUSION: A practice change in transfusion-related adverse reaction reporting is needed to achieve optimal hemovigilance at this institution. Using the barriers and facilitators identified in this study, institutions can better inform future interventions by employing strategies like TR reporting education in order to improve reporting of transfusion-related adverse reactions in this hospital and other similar institutions. RELEVANCE TO CLINICAL PRACTICE: This study informs clinical practice and decision-making for nurses and nursing educators who manage blood transfusion administration procedures.
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Enfermeras y Enfermeros , Reacción a la Transfusión , Adulto , Humanos , Docentes de Enfermería , Canadá , Lista de VerificaciónRESUMEN
BACKGROUND AND OBJECTIVES: Plasma is often transfused to patients with bleeding or requiring invasive procedures and with abnormal tests of coagulation. Chart audits find half of plasma transfusions unnecessary, resulting in avoidable complications and costs. This multicentre electronic audit was conducted to determine the proportion of plasma transfused without an indication and/or at a sub-therapeutic dose. METHODS: Data were extracted on adult inpatients in 2017 at five academic sites from the hospital electronic chart, laboratory information systems and the Canadian Institute for Health Information Discharge Abstract Database. Electronic criteria for plasma transfusion outside recommended indications were: (1) international normalized ratio (INR) < 1.5 with no to moderate bleeding; (2) INR ≥ 1.5, with no to mild bleeding and no planned procedures; and (3) no INR before or after plasma infusion. Sub-therapeutic dose was defined as ≤2 units transfused. RESULTS: In 1 year, 2590 patients received 6088 plasma transfusions encompassing 11,490 units of plasma occurred at the five sites. 77.7% of events were either outside indications or under-dosed. Of these, 34.8% of plasma orders had no indication identified, and 62% of these occurred in non-bleeding patients and no planned procedure with an isolated elevated INR. 70.7% of transfusions were under-dosed. Most plasma transfusions occurred in the intensive care unit or the operating room. Inter-hospital variability in peri-transfusion testing and dosing was observed. CONCLUSION: The majority of plasma transfusions are sub-optimal. Local hospital culture may be an important driver. Electronic audits, with definitions employed in this study, may be a practical alternative to costly chart audits.
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Transfusión de Componentes Sanguíneos , Plasma , Adulto , Transfusión de Componentes Sanguíneos/métodos , Canadá , Electrónica , Hemorragia , Humanos , Relación Normalizada InternacionalRESUMEN
Graft versus host disease is a rare but deadly complication of solid organ transplant. Clinical features of graft-versus-host-disease are non-specific, which may lead to delayed diagnosis as more common conditions including infections or drug reactions are considered. We describe a 54-year-old male patient who underwent liver transplantation for alcohol use disorder-related cirrhosis and developed acute graft-versus-host disease. Initial clinical presentation included dermatitis, bone marrow failure and enteritis. Results of skin biopsy and cytogenetic studies were consistent with liver transplant-associated acute graft-versus-host disease. The importance of this case is to highlight to transplant physicians and surgeons the challenges of diagnosing graft-versus-host-disease. In our case, pre-existing partnerships among the liver and hematopoietic stem cell transplant teams, transfusion medicine specialists, critical care specialists and facilitated timely communication relevant to confirming graft-versus-host disease. We propose an algorithm to assist in the workup of suspected graft-versus-host disease. Because this condition is characterized by high mortality, a high index of suspicion is imperative for prompt diagnosis and optimal management of the donor-recipient immune interaction when patients present with classic clinical features.
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Enfermedad Injerto contra Huésped , Trasplante de Hígado , Enfermedad Injerto contra Huésped/diagnóstico , Enfermedad Injerto contra Huésped/etiología , Humanos , Trasplante de Hígado/efectos adversos , Masculino , Persona de Mediana Edad , Linfocitos TRESUMEN
INTRODUCTION: Packed red blood cell (RBC) transfusion is frequently used in patients undergoing radiotherapy (RT) because retrospective data suggest that anemic patients may respond sub-optimally to RT. No high-quality evidence currently exists to guide transfusion practices and establish hemoglobin (Hb) transfusion thresholds for this patient population, and practice varies significantly across centers. This systematic review investigated whether maintaining higher Hb via transfusion in radiation oncology patients leads to improved outcomes. METHODS: We performed a literature search of studies comparing RBC transfusion thresholds in radiation oncology patients. Included studies assessed patients receiving RT for malignancy of any diagnosis or stage. Excluded studies did not evaluate Hb or transfusion as an intervention or outcome. The primary outcome was overall survival. Secondary outcomes included locoregional control, number of transfusions and adverse events. RESULTS: One study met inclusion criteria. The study pooled results from two randomized controlled trials that stratified anemic patients with head and neck squamous cell carcinoma to RBC transfusion versus no transfusion. The study found no significant differences in overall survival or locoregional control after five years, despite increased Hb levels in the transfused group. We conducted a narrative review by extracting data from 10 non-comparative studies involving transfusion in patients receiving RT. Results demonstrated no consistent conclusions regarding whether transfusions improve or worsen outcomes. CONCLUSIONS: There is a lack of data on the effects of RBC transfusion on outcomes in patients undergoing RT. Well-designed prospective studies are needed in this area.
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BACKGROUND: The relationship between ABO non-identical transfusion and the outcomes of necrotizing enterocolitis (NEC), and all-cause mortality in very-low birth weight (VLBW) neonates receiving red blood cell transfusion is unknown. STUDY DESIGN AND METHODS: A retrospective multicenter cohort study was conducted in VLBW neonates in neonatal intensive care units between 2004 and 2016. VLBW (≤1500 grams) neonates were followed until discharge or in-hospital death. The primary exposure was ABO group. Secondary exposures included platelet count, plasma transfusions, and maternal ABO group. Outcome measures were NEC (defined as Bell stage ≥ 2) and all-cause mortality. Time-dependent Cox regression models with competing risks were used to investigate factors associated with NEC and mortality. RESULTS: Thousand and sixteen neonates were included with 10.8% developing NEC (n = 110) and 14.1% mortality (n = 143). Platelet count (hazard ratio [HR] = 0.995; 95% confidence interval [CI]: 0.922-0.998) and number of plasma transfusions (HR = 2.908; 95% CI:1.265-6.682) were associated with NEC, while ABO group (non-O vs. O) was not (HR = 0.761; 95% CI: 0.393-1.471). Higher all-cause mortality occurred in neonates without NEC who were non-O compared with O (HR = 17.5; 95% CI: 1.784-171.692), but not in neonates with NEC (HR = 1.112; 95% CI: 0.142-8.841). Plasma transfusion was associated with increased mortality in both groups. DISCUSSION: ABO non-identical transfusion was not associated with NEC or mortality in neonates with NEC. It was associated with increased mortality in neonates without NEC. As many neonatal intensive care units transfuse only O group blood as routine practice, future trials are needed to investigate the association between this practice and neonatal mortality.
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Enterocolitis Necrotizante , Enfermedades del Recién Nacido , Transfusión de Componentes Sanguíneos/efectos adversos , Estudios de Cohortes , Enterocolitis Necrotizante/etiología , Edad Gestacional , Mortalidad Hospitalaria , Humanos , Recién Nacido , Recién Nacido de muy Bajo Peso , Plasma , Estudios Retrospectivos , Factores de RiesgoAsunto(s)
Anemia Hemolítica Autoinmune , Anticuerpos Monoclonales Humanizados , Enfermedad de Crohn , Anemia Hemolítica Autoinmune/inducido químicamente , Anticuerpos Monoclonales Humanizados/efectos adversos , Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/tratamiento farmacológico , Humanos , Inmunoglobulina ARESUMEN
BACKGROUND: Study-level meta-analyses provide high-certainty evidence that heparin reduces the risk of symptomatic venous thromboembolism for patients with cancer; however, whether the benefits and harms associated with heparin differ by cancer type is unclear. This individual participant data meta-analysis of randomised controlled trials examines the effect of heparin on survival, venous thromboembolism, and bleeding in patients with cancer in general and by type. METHODS: In this systematic review and meta-analysis we searched MEDLINE, Embase, and The Cochrane Library for randomised controlled trials comparing parenteral anticoagulants with placebo or standard care in ambulatory patients with solid tumours and no indication for anticoagulation published from the inception of each database to January 14, 2017, and updated it on May 14, 2020, without language restrictions. We calculated the effect of parenteral anticoagulant administration on all-cause mortality, venous thromboembolism occurrence, and bleeding related outcomes through multivariable hierarchical models with patient-level variables as fixed effects and a categorical trial variable as a random effect, adjusting for age, cancer type, and metastatic status. Interaction terms were tested to investigate effects in predefined subgroups. This study is registered with PROSPERO, CRD42013003526. FINDINGS: We obtained individual participant data from 14 of 20 eligible randomised controlled trials (8278 [79%] of 10â431 participants; 4139 included in the low-molecular-weight heparin group and 4139 in the control group). Meta-analysis showed an adjusted relative risk (RR) of mortality at 1 year of 0·99 (95% CI 0·93-1·06) and a hazard ratio of 1·01 (95% CI 0·96-1·07). The number of patients with venous thromboembolic events was 158 (4·0%) of 3958 with available data in the low-molecular-weight heparin group compared with 279 (7·1%) of 3957 in the control group. Major bleeding events occurred in 71 (1·7%) of 4139 patients in the control population and 88 (2·1%) in the low-molecular-weight heparin group, and minor bleeding events in 478 (12·1%) of 3945 patients with available data in the control group and 652 (16·6%) of 3937 patients in the low-molecular-weight heparin group. The adjusted RR was 0·58 (95% CI 0·47-0·71) for venous thromboembolism, 1·27 (0·92-1·74) for major bleeding, and 1·34 (1·19-1·51) for minor bleeding. Prespecified subgroup analysis of venous thromboembolism occurrence by cancer type identified the most certain benefit from heparin treatment in patients with lung cancer (RR 0·59 [95% CI 0·42-0·81]), which dominated the overall reduction in venous thromboembolism. Certainty of the evidence for the outcomes ranged from moderate to high. INTERPRETATION: Low-molecular-weight heparin reduces risk of venous thromboembolism without increasing risk of major bleeding compared with placebo or standard care in patients with solid tumours, but it does not improve survival. FUNDING: Canadian Institutes of Health Research.
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Anticoagulantes/uso terapéutico , Heparina/uso terapéutico , Neoplasias/complicaciones , Tromboembolia Venosa/etiología , Tromboembolia Venosa/prevención & control , Anticoagulantes/efectos adversos , Hemorragia/inducido químicamente , Heparina/efectos adversos , Heparina de Bajo-Peso-Molecular/efectos adversos , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Análisis de SupervivenciaRESUMEN
BACKGROUND: Disseminated intravascular coagulation (DIC), a systemic activation of coagulation, presents with multiple clinical and laboratory manifestations. In this International Society on Thrombosis and Haemostasis (ISTH) communication, we examined the importance of identifying the underlying disorder causing DIC to help physicians in the diagnosis and management of this common and severe condition. METHODS: Eight DIC experts participated in a three-step consensus process that searched for published guidelines and diagnostic scores on DIC to create a preliminary list of DIC underlying disorders from those reported in the literature Overall, 13 papers were identified, including three guidelines, one harmonization paper by the ISTH, one ISTH recommendation paper on cancer-associated DIC, five general diagnostic scores, two scores specific for pregnancy, and one specific for children. We then assessed the strength of the evidence on the association between the disease and DIC as many postulated DIC-associated disorders are rare. KEY RESULTS: Eight main subgroups - 'severe infection', 'solid tumour', 'haematological neoplasia', 'pregnancy complication', 'vascular disease', 'newborn-complication', 'tissue damage due to internal or external insult', and 'chemical and biological agent' - and a detailed list of specific causes of DIC were provided. CONCLUSIONS & INFERENCES: Our results suggest more data are needed to determine the association between DIC and specific diseases such as malignant lymphoma, colorectal cancer, or vasculitis, for which the evidence remains limited. When a patient develops a coagulopathy consistent with DIC, the first step is to immediately search for an underlying disorder, including specific causes that are rarely associated with DIC and to consider that patients may have more than one cause of DIC to identify the principal precipitating disorder to prioritize treatment.
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Coagulación Intravascular Diseminada , Trombosis , Niño , Comunicación , Cuidados Críticos , Coagulación Intravascular Diseminada/diagnóstico , Femenino , Hemostasis , Humanos , Recién Nacido , EmbarazoRESUMEN
BACKGROUND: Sickle cell disease (SCD) is associated with hematologic complications including delayed hemolytic transfusion reactions (DHTRs) and pregnancy-related morbidity and mortality. Hyperhemolysis syndrome (HS) is the most severe form of DHTR in patients with SCD, in which both transfused and native red blood cells are destroyed. Further transfusions are avoided after a history of HS. Immunosuppressive agents can be used as prophylaxis against life-threatening hemolysis when transfusion is necessary. There is a paucity of evidence for the use of HS prophylaxis before transfusions, the continuation of hydroxyurea (HU) in lieu of chronic transfusion, and the use of erythropoiesis-stimulating agents (ESA) in pregnant SCD patients. CASE REPORT: We present a case of a pregnant patient with SCD and a previous history of HS. HS prophylaxis was given before transfusion with corticosteroids, intravenous immunoglobulin, and rituximab. In addition, HU was continued during pregnancy to control SCD, along with the use of concomitant ESA to maintain adequate hemoglobin levels and avoid transfusion. We describe a multidisciplinary approach to pregnancy and delivery management including tailored anesthetic and obstetric planning. CONCLUSION: This is the first published case of HS prophylaxis in a pregnant SCD patient, with good maternal and fetal outcomes after transfusion. HU and ESAs were able to control SCD and mitigate anemia in lieu of prophylactic transfusions during pregnancy. Further prospective studies are necessary to elucidate the ideal management of pregnant SCD patients with a history of HS or other contraindications to chronic transfusion.
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Corticoesteroides/administración & dosificación , Anemia de Células Falciformes , Hemólisis/efectos de los fármacos , Inmunoglobulinas Intravenosas/administración & dosificación , Periodo Periparto/sangre , Complicaciones Hematológicas del Embarazo , Rituximab/administración & dosificación , Reacción a la Transfusión , Adulto , Anemia de Células Falciformes/sangre , Anemia de Células Falciformes/tratamiento farmacológico , Femenino , Humanos , Embarazo , Complicaciones Hematológicas del Embarazo/sangre , Complicaciones Hematológicas del Embarazo/tratamiento farmacológico , Síndrome , Reacción a la Transfusión/sangre , Reacción a la Transfusión/prevención & controlRESUMEN
BACKGROUND: Oncology guidelines suggest using the Khorana score to select ambulatory cancer patients receiving chemotherapy for primary venous thromboembolism (VTE) prevention, but its performance in different cancers remains uncertain. OBJECTIVE: To examine the performance of the Khorana score in assessing 6-month VTE risk, and the efficacy and safety of low-molecular-weight heparin (LMWH) among high-risk Khorana score patients. METHODS: This individual patient data meta-analysis evaluated (ultra)-LMWH in patients with solid cancer using data from seven randomized controlled trials. RESULTS: A total of 3293 patients from the control groups with an available Khorana score had lung (n = 1913; 58%), colorectal (n = 452; 14%), pancreatic (n = 264; 8%), gastric (n = 201; 6%), ovarian (n = 184; 56%), breast (n = 164; 5%), brain (n = 84; 3%), or bladder cancer (n = 31; 1%). The 6-month VTE incidence was 9.8% among high-risk Khorana score patients and 6.4% among low-to-intermediate-risk patients (odds ratio [OR], 1.6; 95% confidence interval [CI], 1.1-2.2). The dichotomous Khorana score performed differently in lung cancer patients (OR 1.1; 95% CI, 0.72-1.7) than in the group with other cancer types (OR 3.2; 95% CI, 1.8-5.6; Pinteraction = .002). Among high-risk patients, LMWH decreased the risk of VTE by 64% compared with controls (OR 0.36; 95% CI, 0.22-0.58), without increasing the risk of major bleeding (OR 1.1; 95% CI, 0.59-2.1). CONCLUSION: The Khorana score was unable to stratify patients with lung cancer based on their VTE risk. Among those with other cancer types, a high-risk score was associated with a three-fold increased risk of VTE compared with a low-to-intermediate risk score. Thromboprophylaxis was effective and safe in patients with a high-risk Khorana score.
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Neoplasias , Tromboembolia Venosa , Anticoagulantes/efectos adversos , Hemorragia , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Neoplasias/complicaciones , Neoplasias/tratamiento farmacológico , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/epidemiología , Tromboembolia Venosa/etiologíaRESUMEN
BACKGROUND: The efficacy of premedication for the prevention of nonhemolytic transfusion reactions remains controversial. This systematic review and meta-analysis assessed the effect of premedication on the rate of nonhemolytic transfusion reactions after allogeneic blood transfusion. STUDY DESIGN AND METHODS: We searched the literature using CENTRAL, MEDLINE, EMBASE, ISI Web of Science, and clinicaltrials.gov databases from inception until October 31, 2018. We included all randomized controlled trials comparing premedication to placebo or no treatment in patients receiving any labile blood product. Outcome measures were reported as relative risks (RRs) with 95% confidence intervals (CIs). Data were combined for similar outcomes where appropriate using a random-effects model. Analyses were done at both the patient and transfusion level. RESULTS: Three randomized trials using acetaminophen and antihistamine as premedication met the inclusion criteria. A total of 517 patients received 4444 red blood cell or platelet transfusions. Pooled patient-level estimates with premedication for all nonhemolytic, febrile nonhemolytic, and minor allergic reactions were RR, 0.92 (95% CI, 0.63-1.35); RR, 0.54 (95% CI, 0.26-1.1); and RR, 1.37 (95% CI 0.81-2.31), respectively. Transfusion-level analyses also showed no benefit with premedication. Of 517 patients randomized, only 27 (5.2%) had a history of transfusion reactions. CONCLUSION: Routine premedication with acetaminophen and antihistamines did not prevent nonhemolytic transfusion reactions; however, the estimate of effect was greatest for febrile reactions. The impact of premedication in patients with a prior history of transfusion reactions remains unknown and requires further evaluation in future clinical trials.
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Premedicación/métodos , Reacción a la Transfusión/prevención & control , Acetaminofén/uso terapéutico , Transfusión Sanguínea , Antagonistas de los Receptores Histamínicos/uso terapéutico , Humanos , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
We present the case of a novel ß-globin gene variant associated with early-onset transfusion-dependent anemia compatible with a ß-thalassemia major (ß-TM) phenotype in a patient of British descent. As a child, our patient developed chronic symptomatic anemia with hemoglobin (Hb) nadirs of 3.0 g/dL. She started receiving occasional transfusions by the age of 13 years and became transfusion-dependent by the age of 32 years. Work-up performed at our center showed a Hb electrophoresis compatible with ß-thalassemia (ß-thal) trait. Polymerase chain reaction (PCR) of the ß-globin gene detected a novel mutation situated at codon 110 (CTG). This missense mutation led to a substitution of the thymine nucleotide (nt) base for guanine (CGG) at position 332 (HBB: c.332T>G). We have named this new mutation Hb London-Ontario. The majority of previously described dominant allelic mutations of the ß-globin gene led to a ß-thal intermedia (ß-TI) phenotype. The heterozygous mutation which was detected in our patients is unique at it leads to a more severe ß-TM phenotype. We suspect this is a de novo mutation of which the mother of our patient, who was reported to have a form of thalassemia, was the proband.