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Pathophysiology of Sickle Cell Disease Open
Since the discovery of sickle cell disease (SCD) in 1910, enormous strides have been made in the elucidation of the pathogenesis of its protean complications, which has inspired recent advances in targeted molecular therapies. In SCD, a si…
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Intravascular hemolysis and the pathophysiology of sickle cell disease Open
Hemolysis is a fundamental feature of sickle cell anemia that contributes to its pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1, asymmetric dimethylarginine, and adenine nucleotides are all products …
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Blood-Contacting Biomaterials: In Vitro Evaluation of the Hemocompatibility Open
Hemocompatibility of blood-contacting biomaterials is one of the most important criteria for their successful in vivo applicability. Thus, extensive in vitro analyses according to ISO 10993-4 are required prior to clinical applications. In…
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Ravulizumab (ALXN1210) vs eculizumab in adult patients with PNH naive to complement inhibitors: the 301 study Open
Ravulizumab (ALXN1210), a new complement C5 inhibitor, provides immediate, complete, and sustained C5 inhibition. This phase 3, open-label study assessed the noninferiority of ravulizumab to eculizumab in complement inhibitor–naive adults …
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Ravulizumab (ALXN1210) vs eculizumab in C5-inhibitor–experienced adult patients with PNH: the 302 study Open
Ravulizumab, a new complement component C5 inhibitor administered every 8 weeks, was noninferior to eculizumab administered every 2 weeks in complement-inhibitor–naive patients with paroxysmal nocturnal hemoglobinuria (PNH). This study ass…
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Glucose-6-phosphate dehydrogenase deficiency Open
Glucose 6-phosphate dehydrogenase (G6PD) deficiency is 1 of the commonest human enzymopathies, caused by inherited mutations of the X-linked gene G6PD. G6PD deficiency makes red cells highly vulnerable to oxidative damage, and therefore su…
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Persistent complement dysregulation with signs of thromboinflammation in active Long Covid Open
Long Covid is a debilitating condition of unknown etiology. We performed multimodal proteomics analyses of blood serum from COVID-19 patients followed up to 12 months after confirmed severe acute respiratory syndrome coronavirus 2 infectio…
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Optimization of the Hemolysis Assay for the Assessment of Cytotoxicity Open
In vitro determination of hemolytic properties is a common and important method for preliminary evaluation of cytotoxicity of chemicals, drugs, or any blood-contacting medical device or material. The method itself is relatively straightfor…
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In Vitro Anti-Inflammatory Properties of Selected Green Leafy Vegetables Open
The study investigated the anti-inflammatory activity of the hydro methanolic extract of six leafy vegetables, namely Cassia auriculata, Passiflora edulis, Sesbania grandiflora, Olax zeylanica, Gymnema lactiferum, and Centella asiatica. Th…
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An Evaluation of Blood Compatibility of Silver Nanoparticles Open
Silver nanoparticles (AgNPs) have tremendous potentials in medical devices due to their excellent antimicrobial properties. Blood compatibility should be investigated for AgNPs due to the potential blood contact. However, so far, most stud…
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Membrane stabilization as a mechanism of the anti-inflammatory activity of ethanolic root extract of Choi (Piper chaba) Open
Background This experiment is conducted to evaluate the anti-inflammatory effect of Piper chaba roots. Methods The in-vitro anti-inflammatory activity of Piper chaba was carried out by human red blood cell (HRBC) membrane stabilization met…
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Ethnicity, sex, and age are determinants of red blood cell storage and stress hemolysis: results of the REDS-III RBC-Omics study Open
Key Points Genetic and biological variability in blood donors may impact RBC predisposition to hemolysis during cold storage and after transfusion. Male sex, Asian or African American racial background, and older age (>45 years) are signif…
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Comparison of Methodologies to Detect Low Levels of Hemolysis in Serum for Accurate Assessment of Serum microRNAs Open
microRNAs have emerged as powerful regulators of many biological processes, and their expression in many cancer tissues has been shown to correlate with clinical parameters such as cancer type and prognosis. Present in a variety of biologi…
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Small-molecule factor B inhibitor for the treatment of complement-mediated diseases Open
Dysregulation of the alternative complement pathway (AP) predisposes individuals to a number of diseases including paroxysmal nocturnal hemoglobinuria, atypical hemolytic uremic syndrome, and C3 glomerulopathy. Moreover, glomerular Ig depo…
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Role of red blood cells in haemostasis and thrombosis Open
In contrast to an obsolete notion that erythrocytes, or red blood cells ( RBC s), play a passive and minor role in haemostasis and thrombosis, over the past decades there has been increasing evidence that RBC s have biologically and clinic…
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Anti-complement Treatment for Paroxysmal Nocturnal Hemoglobinuria: Time for Proximal Complement Inhibition? A Position Paper From the SAAWP of the EBMT Open
The treatment of paroxysmal nocturnal hemoglobinuria has been revolutionized by the introduction of the anti-C5 agent eculizumab; however, eculizumab is not the cure for Paroxysmal nocturnal hemoglobinuria (PNH), and room for improvement r…
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Prolonged red cell storage before transfusion increases extravascular hemolysis Open
NIH grant HL115557 and UL1 TR000040.
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Sutimlimab in Cold Agglutinin Disease Open
In patients with cold agglutinin disease who received sutimlimab, selective upstream inhibition of activity in the classic complement pathway rapidly halted hemolysis, increased hemoglobin levels, and reduced fatigue. (Funded by Sanofi; CA…
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How I treat autoimmune hemolytic anemia Open
Autoimmune hemolytic anemia (AIHA) is an uncommon entity that presents diagnostic, prognostic, and therapeutic dilemmas despite being a well-recognized entity for over 150 years. This is because of significant differences in the rates of h…
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Pro-inflammatory Actions of Heme and Other Hemoglobin-Derived DAMPs Open
Damage associated molecular patterns (DAMPs) are endogenous molecules originate from damaged cells and tissues with the ability to trigger and/or modify innate immune responses. Upon hemolysis hemoglobin (Hb) is released from red blood cel…
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Intravascular hemolysis activates complement via cell-free heme and heme-loaded microvesicles Open
In hemolytic diseases, such as sickle cell disease (SCD), intravascular hemolysis results in the release of hemoglobin, heme, and heme-loaded membrane microvesicles in the bloodstream. Intravascular hemolysis is thus associated with inflam…
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Novel insights into the treatment of complement-mediated hemolytic anemias Open
Complement-mediated hemolytic anemias can either be caused by deficiencies in regulatory complement components or by autoimmune pathogenesis that triggers inappropriate complement activation. In paroxysmal nocturnal hemoglobinuria (PNH) he…
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Autoimmune hemolytic anemia Open
The diagnosis of autoimmune hemolytic anemia (AIHA) can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin te…
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Hypoxia modulates the purine salvage pathway and decreases red blood cell and supernatant levels of hypoxanthine during refrigerated storage Open
Hypoxanthine catabolism in vivo is potentially dangerous as it fuels production of urate and, most importantly, hydrogen peroxide. However, it is unclear whether accumulation of intracellular and supernatant hypoxanthine in stored red bloo…
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How I safely transfuse patients with sickle-cell disease and manage delayed hemolytic transfusion reactions Open
Transfusions can be a life-saving treatment of patients with sickle-cell disease (SCD). However, availability of matched units can be limiting because of distinctive blood group polymorphisms in patients of African descent. Development of …
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Donor sex, age and ethnicity impact stored red blood cell antioxidant metabolism through mechanisms in part explained by glucose 6-phosphate dehydrogenase levels and activity Open
Red blood cell storage in the blood bank promotes the progressive accumulation of metabolic alterations that may ultimately impact the erythrocyte capacity to cope with oxidant stressors. However, the metabolic underpinnings of the capacit…
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Antimicrobial Activity of Quercetin, Naringenin and Catechin: Flavonoids Inhibit Staphylococcus aureus-Induced Hemolysis and Modify Membranes of Bacteria and Erythrocytes Open
Search for novel antimicrobial agents, including plant-derived flavonoids, and evaluation of the mechanisms of their antibacterial activities are pivotal objectives. The goal of this study was to compare the antihemolytic activity of flavo…
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Erythrocytic ferroportin reduces intracellular iron accumulation, hemolysis, and malaria risk Open
Iron's grip on malaria Malaria parasites have coevolved with their human and mammalian hosts. These Plasmodium species invade the iron-rich environment of red blood cells. Zhang et al. found that the iron transporter ferroportin persists o…
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New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia Open
Autoimmune hemolytic anemias mediated by cold agglutinins can be divided into cold agglutinin disease (CAD), which is a well-defined clinicopathologic entity and a clonal lymphoproliferative disorder, and secondary cold agglutinin syndrome…
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Testosterone‐dependent sex differences in red blood cell hemolysis in storage, stress, and disease Open
BACKGROUND Red blood cell (RBC) hemolysis represents an intrinsic mechanism for human vascular disease. Intravascular hemolysis releases hemoglobin and other metabolites that inhibit nitric oxide signaling and drive oxidative and inflammat…