-
Journal of Perinatology : Official... Feb 2023The diagnosis of ABO hemolytic disease of the newborn (ABO HDN) has been the subject of considerable debate and clinical confusion. Its use as an overarching default... (Review)
Review
The diagnosis of ABO hemolytic disease of the newborn (ABO HDN) has been the subject of considerable debate and clinical confusion. Its use as an overarching default diagnosis for hyperbilirubinemia in all ABO incompatible neonates regardless of serological findings is problematic and lacks diagnostic precision. Data on hemolysis indexed by carbon monoxide (CO) levels in expired air (ETCOc) and blood (COHbc) support an essential role for a positive direct antiglobulin test (DAT) in making a more precise diagnosis of ABO HDN. A working definition that includes ABO incompatibility, significant neonatal hyperbilirubinemia, and a positive DAT is needed to gain clarity and consistency in the diagnosis of ABO HDN. Absent a positive DAT, the diagnosis of ABO HDN is suspect. Instead, a negative DAT in a severely hyperbilirubinemic ABO incompatible neonate should trigger an exhaustive search for an alternative cause, a search that may require the use of targeted gene panels.
Topics: Infant, Newborn; Female; Humans; ABO Blood-Group System; Erythroblastosis, Fetal; Blood Group Incompatibility; Hyperbilirubinemia, Neonatal; Hemolysis; Coombs Test
PubMed: 36344813
DOI: 10.1038/s41372-022-01556-6 -
Blood Advances Jun 2019The terminology applied to autoimmune hemolytic anemia (AIHA) seems inconsistent. We aimed to evaluate the consistency of definitions used for diagnosis and treatment.... (Comparative Study)
Comparative Study
The terminology applied to autoimmune hemolytic anemia (AIHA) seems inconsistent. We aimed to evaluate the consistency of definitions used for diagnosis and treatment. In this systematic review of literature from January 2006 to December 2015, we assessed heterogeneity in the definition of AIHA and its subtypes, refractory disease, disease phase, severity, criteria for treatment response, and response durability. A Medline search for anemia, hemolytic, autoimmune was supplemented with keyword searches. Main exclusions were conference abstracts, animal and non-English studies, and studies with <10 cases. Of 1371 articles retrieved, 1209 were excluded based on titles and abstracts. Two authors independently reviewed 10% and 16% of abstracts and full papers, respectively. After full-paper review, 84 studies were included. AIHA was most frequently (32 [52%] of 61) defined as hemolytic anemia with positive direct antiglobulin test (DAT) and exclusion of alternatives, but 10 of 32 also recognized DAT-negative AIHA. A lower threshold for diagnosis of DAT-negative AIHA was observed in literature on chronic lymphocytic leukemia. Definitions of anemia, hemolysis, and exclusion criteria showed substantial variation. Definitions of primary/secondary cold agglutinin disease/syndrome were not consistent. Forty-three studies provided criteria for treatment response, and other than studies from 1 center, these were almost entirely unique. Other criteria were rarely defined. Only 7, 0, 3, 2, 2, and 3 studies offered definitions of warm AIHA, paroxysmal cold hemoglobinuria, mixed AIHA, AIHA severity, disease phase, and refractory AIHA, respectively. Marked heterogeneity in the time period sampled indicates the need to standardize AIHA terminology.
Topics: Anemia, Hemolytic, Autoimmune; Coombs Test; Erythrocytes; Hemoglobinuria, Paroxysmal; Hemolysis; Humans; Immunoglobulin G; Publications; Severity of Illness Index; Terminology as Topic
PubMed: 31235526
DOI: 10.1182/bloodadvances.2019000036 -
Cureus May 2023Drug-induced hemolytic anemia is rare and can occur either by an immune-mediated mechanism or a non-immune-mediated mechanism. The drugs most frequently associated...
Drug-induced hemolytic anemia is rare and can occur either by an immune-mediated mechanism or a non-immune-mediated mechanism. The drugs most frequently associated with immune-mediated hemolysis are penicillins and cephalosporins. It is usually difficult to distinguish drug-induced hemolysis from other more common causes of hemolysis; therefore, a high index of clinical suspicion is required to make the diagnosis. In this case report, we present a case of vancomycin-induced immune hemolytic anemia in a 75-year-old patient who developed hemolytic anemia after starting vancomycin for joint infection. Hematological parameters improved after the discontinuation of vancomycin. Mechanism and management of drug-induced immune hemolytic anemia are also reviewed in this report.
PubMed: 37216133
DOI: 10.7759/cureus.39191 -
Pediatric Nephrology (Berlin, Germany) Feb 2022
Topics: Escherichia coli Infections; Female; Hemolytic-Uremic Syndrome; Humans; Male; Prognosis; Shiga-Toxigenic Escherichia coli
PubMed: 34796390
DOI: 10.1007/s00467-021-05355-7 -
Annals of Palliative Medicine Apr 2022This study verified and assessed 26 biochemical indicators tested by a dry chemistry analyzer using the hemolytic index test function to determine the degree of...
BACKGROUND
This study verified and assessed 26 biochemical indicators tested by a dry chemistry analyzer using the hemolytic index test function to determine the degree of interference and the trends among the hemolysis samples on the test results. This study also sought to ensure that reasonable test reports could be issued taking into account practical clinical needs.
METHODS
The samples were manually divided into the control group and the test group. The hemolytic index and biochemical indicators of the samples were tested using the Ortho Vitros 5600 to compare the deviation of the test results between the 2 groups. The judgment standard was set as 1/3 of the total error allowable as required by the quality assessment criterion of the National Center for Clinical Laboratories. The interference degree of hemolysis on the dry chemistry-based biochemical indicators was assessed, and the hemolytic thresholds of 26 biochemical indicators provided by the manufacturer were verified in terms of their validity and rationality.
RESULTS
The hemolytic thresholds of 26 dry chemistry-based biochemical indicators were verified to analyze the degree of interference. The results revealed that hemolysis interfered with 17 indicators. Hemolysis positively interfered with the test results of phosphorus, creatine kinase, gamma glutamyl transpeptidase (γ-GGT), magnesium, iron, total protein, potassium, total bilirubin, lactate dehydrogenase, albumin, and aspartate aminotransferase, but negatively interfered with cholinesterase, direct high-density lipoprotein cholesterol, glucose, elevated carbon dioxide alkaline phosphatase, and alanine aminotransferase. A negative deviation of γ-GGT by hemoglobin was described in the manufacturer's statement, but our test data showed a positive deviation by hemolysis. The hemolytic threshold verification results of the other biochemical indicators were consistent with the manufacturer's statement.
CONCLUSIONS
The hemolytic index test function was used to determine which samples were interfered with by hemolysis to make an analytical judgment according to the hemolytic interference thresholds of the different test items, verify the validity of the hemolytic thresholds of the test items, perform reasonable tests on the hemolytic samples, and issue valid reports to reduce the rejection rate of the hemolytic samples, shorten the turnaround time (TAT) of laboratories.
Topics: Aspartate Aminotransferases; Bilirubin; Hemoglobins; Hemolysis; Humans; L-Lactate Dehydrogenase
PubMed: 35523746
DOI: 10.21037/apm-22-292 -
Lancet (London, England) Oct 2008Hereditary spherocytosis is a common inherited disorder that is characterised by anaemia, jaundice, and splenomegaly. It is reported worldwide and is the most common...
Hereditary spherocytosis is a common inherited disorder that is characterised by anaemia, jaundice, and splenomegaly. It is reported worldwide and is the most common inherited anaemia in individuals of northern European ancestry. Clinical severity is variable with most patients having a well-compensated haemolytic anaemia. Some individuals are asymptomatic, whereas others have severe haemolytic anaemia requiring erythrocyte transfusion. The primary lesion in hereditary spherocytosis is loss of membrane surface area, leading to reduced deformability due to defects in the membrane proteins ankyrin, band 3, beta spectrin, alpha spectrin, or protein 4.2. Many isolated mutations have been identified in the genes encoding these membrane proteins; common hereditary spherocytosis-associated mutations have not been identified. Abnormal spherocytes are trapped and destroyed in the spleen and this is the main cause of haemolysis in this disorder. Common complications are cholelithiasis, haemolytic episodes, and aplastic crises. Splenectomy is curative but should be undertaken only after careful assessment of the risks and benefits.
Topics: Gallbladder Diseases; Hemolysis; Humans; Severity of Illness Index; Spherocytosis, Hereditary; Splenectomy
PubMed: 18940465
DOI: 10.1016/S0140-6736(08)61588-3 -
The Medical Clinics of North America Mar 2017Red blood cell (RBC) destruction can be secondary to intrinsic disorders of the RBC or to extrinsic causes. In the congenital hemolytic anemias, intrinsic RBC enzyme,... (Review)
Review
Red blood cell (RBC) destruction can be secondary to intrinsic disorders of the RBC or to extrinsic causes. In the congenital hemolytic anemias, intrinsic RBC enzyme, RBC membrane, and hemoglobin disorders result in hemolysis. The typical clinical presentation is a patient with pallor, anemia, jaundice, and often splenomegaly. The laboratory features include anemia, hyperbilirubinemia, and reticulocytosis. For some congenital hemolytic anemias, splenectomy is curative. However, in other diseases, avoidance of drugs and toxins is the best therapy. Supportive care with transfusions are also mainstays of therapy. Chronic hemolysis often results in the formation of gallstones, and cholecystectomy is often indicated.
Topics: Anemia, Hemolytic, Congenital; Anemia, Hemolytic, Congenital Nonspherocytic; Erythrocyte Membrane; Glucosephosphate Dehydrogenase Deficiency; Hematologic Tests; Hemoglobinopathies; Humans; Pyruvate Kinase; Pyruvate Metabolism, Inborn Errors; Severity of Illness Index; Splenectomy
PubMed: 28189176
DOI: 10.1016/j.mcna.2016.09.008 -
Blood Sep 2020The mature red blood cell (RBC) lacks a nucleus and organelles characteristic of most cells, but it is elegantly structured to perform the essential function of... (Review)
Review
The mature red blood cell (RBC) lacks a nucleus and organelles characteristic of most cells, but it is elegantly structured to perform the essential function of delivering oxygen and removing carbon dioxide from all other cells while enduring the shear stress imposed by navigating small vessels and sinusoids. Over the past several decades, the efforts of biochemists, cell and molecular biologists, and hematologists have provided an appreciation of the complexity of RBC membrane structure, while studies of the RBC membrane disorders have offered valuable insights into structure-function relationships. Within the last decade, advances in genetic testing and its increased availability have made it possible to substantially build upon this foundational knowledge. Although disorders of the RBC membrane due to altered structural organization or altered transport function are heterogeneous, they often present with common clinical findings of hemolytic anemia. However, they may require substantially different management depending on the underlying pathophysiology. Accurate diagnosis is essential to avoid emergence of complications or inappropriate interventions. We propose an algorithm for laboratory evaluation of patients presenting with symptoms and signs of hemolytic anemia with a focus on RBC membrane disorders. Here, we review the genotypic and phenotypic variability of the RBC membrane disorders in order to raise the index of suspicion and highlight the need for correct and timely diagnosis.
Topics: Anemia, Hemolytic; Blood Proteins; Body Water; Cytoskeleton; Desiccation; Erythrocyte Membrane; Erythrocytes, Abnormal; Genetic Association Studies; Humans; Ion Channels; Models, Molecular; Mutation; Protein Conformation; Structure-Activity Relationship
PubMed: 32702754
DOI: 10.1182/blood.2019000946 -
American Journal of Clinical Pathology Jun 2022In vitro hemolysis generates a spurious increase in potassium. Roche Diagnostics recently revised its recommended guidelines for potassium reporting on cobas analyzers....
OBJECTIVES
In vitro hemolysis generates a spurious increase in potassium. Roche Diagnostics recently revised its recommended guidelines for potassium reporting on cobas analyzers. By dramatically reducing the allowable degree of hemolysis, these guidelines would increase specimen rejection rates. We attempted to balance the desire to avoid inaccurate results with the clinical implications of increased specimen rejection rates.
METHODS
We downloaded hemolytic indices (HI) for 80,795 specimens tested at our institution on cobas chemistry analyzers in 1 month and evaluated potential specimen rejection rates based on the new criteria. We also spiked nonhemolyzed samples with hemolyzed blood to assess the influence of HI values on potassium measurements.
RESULTS
The new recommendations would lead to specimen rejection rates of 76% in the neonatal intensive care unit (NICU), 41% in the emergency department (ED), 16% in inpatient specimens, and 9% in outpatient samples. Our current criteria of reporting potassium concentrations in inpatient and outpatient specimens with HI ≤100 and in NICU and ED specimens with HI ≤300 and additional interpretive guidance for HI values between 100 and 300 reduce unnecessary specimen rejections to 3% in NICU, 2% in ED and inpatients, and less than 1% in outpatients without significantly increasing the number of clinically consequential incorrect results.
CONCLUSIONS
The new recommendations would lead to unacceptably high specimen rejection rates. Laboratories should develop context-specific, evidence-based reporting criteria that minimize reporting of inaccurate results without disrupting delivery of care.
Topics: Hematologic Tests; Hemolysis; Humans; Infant, Newborn; Laboratories; Potassium
PubMed: 35038719
DOI: 10.1093/ajcp/aqab217 -
IEEE Transactions on Bio-medical... Aug 2022In preclinical examinations, rotodynamic blood pumps (RBPs) are predominantly evaluated at design-point conditions. In clinical practice, however, they run at...
OBJECTIVE
In preclinical examinations, rotodynamic blood pumps (RBPs) are predominantly evaluated at design-point conditions. In clinical practice, however, they run at diversified modes of operation. This study aimed at extending current preclinical evaluation of hemolytic profiles in RBPs toward broader, clinically relevant ranges of operation.
METHODS
Two implantable RBPs - the HeartMate 3 (HM3) and the HeartWare Ventricular Assist Device (HVAD) - were analyzed at three pump speeds (HM3: 4300, 5600, 7000 rpm; HVAD: 1800, 2760, 3600 rpm) with three flow rates (1-9L/min) per speed setting. Hemolysis measurements were performed in heparinized bovine blood. The delta free hemoglobin (dfHb) and the normalized index of hemolysis (NIH) served as hemolytic measures. Statistical analysis was performed by multiple comparison of the 9 operating conditions. Moreover, computational fluid dynamics (CFD) was applied to provide mechanistic insights into the interrelation between hydraulics and hemolysis by correlating numerically computed hydraulic losses with in-vitro hemolytic measures.
RESULTS
In both devices, dfHb increased toward increasing speeds, particularly during low but also during high flow condition. By contrast, in both RBPs magnitudes of NIH were significantly elevated during low flow operation compared to high flow conditions (p<0.0036). Maps of hemolytic metrics revealed morphologically similar trends to in-silico hydraulic losses (r>0.793).
CONCLUSIONS
While off-design operation is associated with increased hemolytic profiles, the setting of different operating conditions render a preclinical prediction of clinical impact with current hemolysis metrics difficult.
SIGNIFICANCE
The identified increase in hemolytic measures during episodes of off-design operation is highlighting the need to consider worst-case operation during preclinical examinations.
Topics: Animals; Cattle; Heart-Assist Devices; Hemoglobins; Hemolysis; Hydrodynamics
PubMed: 35085069
DOI: 10.1109/TBME.2022.3146135