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The article discusses a pilot study on the use of low-dose iron chelation as anti-oxidative therapy in patients with sickle cell disease. The study aimed to investigate the potential of iron chelation in reducing oxidative stress and improving various health markers in patients with sickle cell disease. The results showed a decrease in haemolysis markers after treatment, but no significant changes in erythrocyte sickling or adhesion. The study suggests that further research with a larger sample size and longer treatment duration is needed to determine the effectiveness of low-dose iron chelation in managing sickle cell disease. [Extracted from the article]

Objective: The study aimed to determine the impact of Red Blood Cells (RBCs) generated from peripheral blood mononuclear cells (PBMCs) on T cell proliferation and host response following whole blood stimulation. Background: Culturing RBCs is a potential solution for donor shortage. The impact of immature cultured RBCs which express CD71+ on host immune response is not known. Methods/Materials: PBMCs were seeded in an erythroid expansion medium. CD71+ cells were isolated at days 14 and 21 of culture and incubated with either purified T cells or with LPS‐stimulated whole blood. Controls were incubated with medium. Results: At day 9, the percentage of cells that expressed CD45 and CD71 reached to the highest level (32.9%, IQR; 26.2–39.05) while the percentage of cells that expressed CD71 and CD235a reached to the highest level on day 17 (70.2%, IQR; 66.1–72.8). Incubation of T cells with days 14 CD71+ cells and day 21 CD71+ cells increased T cell proliferation. In a whole blood stimulation assay, day 21 CD71+ cells, but not day 14 CD71+ cells, inhibited the production of IL‐6 and TNFα. Conclusion: Cultured erythroid cells can modulate the immune response by promoting T cell proliferation and inhibiting cytokine secretions following whole blood stimulation. [ABSTRACT FROM AUTHOR]

Background: Red blood cell (RBC) transfusion is associated with adverse effects, which may involve activation of the host immune response. The effect of RBC transfusion on neutrophil Reactive Oxygen Species (ROS) production and adhesion ex vivo was investigated in endotoxemic volunteers and in critically ill patients that received a RBC transfusion. We hypothesized that RBC transfusion would cause neutrophil activation, the extent of which depends on the storage time and the inflammatory status of the recipient.Study Design and Methods: Volunteers were injected with lipopolysaccharide (LPS) and transfused with either saline, fresh, or stored autologous RBCs. In addition, 47 critically ill patients with and without sepsis receiving either fresh (<8 days) or standard stored RBC (2-35 days) were included. Neutrophils from healthy volunteers were incubated with the plasma samples from the endotoxemic volunteers and from the critically ill patients, after which priming of neutrophil ROS production and adhesion were assessed.Results: In the endotoxemia model, ex vivo neutrophil adhesion, but not ROS production, was increased after transfusion, which was not affected by RBC storage duration. In the critically ill, ex vivo neutrophil ROS production was already increased prior to transfusion and was not increased following transfusion. Neutrophil adhesion was increased following transfusion, which was more notable in the septic patients than in non-septic patients. Transfusion of fresh RBCs, but not standard issued RBCs, resulted in enhanced ROS production in neutrophils.Conclusion: RBC transfusion was associated with increased neutrophil adhesion in a model of human endotoxemia as well as in critically ill patients with sepsis. [ABSTRACT FROM AUTHOR]

Background: Apheresis is increasingly being applied to collect cells or plasma, even allowing the collection of multiple blood components during one procedure. Although the quality of the cellular and plasma products that are obtained by apheresis have been extensively studied and shown to be of high quality, the impact of apheresis on the red blood cells (RBCs) that are returned to the donor has not been investigated.Study Design and Methods: The effect of the plasma- or plateletpheresis procedures by four different devices-MCS+ (Haemonetics), PCS2 (Haemonetics), Trima Accel (Terumo BCT), and Autopheresis-C (Auto-C, Fresenius Kabi)-on the RBCs that are returned to the donor was tested in a blinded, prospective trial in a cohort of 25 donors.Results: A rheologic analysis of donor RBCs before and after plasma- or plateletpheresis showed no differences in outcome. However, a strong increase in hemolysis was found in samples from the Trima Accel devices after plateletpheresis, compared to all other machines tested. Furthermore, an increase in complement deposition on RBCs was seen after all plasmapheresis procedures (MCS+, PCS2, and Auto-C). Finally, a significant decrease in the expression of the complement-regulating protein CD59 was seen in all postapheresis samples as well as a significant decrease of the adhesion molecule CD147.Conclusion: The increase in complement deposition and the decrease in the expression of CD59 suggests that RBC clearance might be enhanced after return to the donor. Possible side effects due to an increase in hemolysis after Trima Accel plateletpheresis should be further investigated. [ABSTRACT FROM AUTHOR]

Objective Here, we present a 7-year-old patient suffering from severe haemolytic anaemia. The most common cause of chronic hereditary non-spherocytic haemolytic anaemia is red blood cell pyruvate kinase (PK-R) deficiency. Because red blood cells rely solely on glycolysis to generate ATP, PK-R deficiency can severely impact energy supply and cause reduction in red blood cell lifespan. We determined the underlying cause of the anaemia and investigated how erythroid precursors in the patient survive. Methods PK activity assays, Western blot and Sanger sequencing were employed to determine the underlying cause of the anaemia. Patient erythroblasts were cultured and reticulocytes were isolated to determine PK-R and PKM2 contribution to glycolytic activity during erythrocyte development. Results We found a novel homozygous mutation (c.583G>A) in the PK-R coding gene ( PKLR). Although this mutation did not influence PKLR mRNA production, no PK-R protein could be detected in the red blood cells nor in its precursors. In spite of the absence of PK-R, the reticulocytes of the patient exhibited 20% PK activity compared with control. Western blotting revealed that patient erythroid precursors, like controls, express residual PKM2. Conclusions We conclude that PKM2 rescues glycolysis in PK-R-deficient erythroid precursors. [ABSTRACT FROM AUTHOR]