The energy-less red blood cell is lost: erythrocyte enzyme abnormalities of glycolysis

The red blood cell depends solely on the anaerobic conversion of glucose by the Embden-Meyerhof pathway for the generation and storage of high-energy phosphates, which is necessary for the maintenance of a number of vital functions. Many red blood cell enzymopathies have been described that disturb the erythrocyte's integrity, shorten its cellular survival, and result in hemolytic anemia. By far the majority of these enzymopathies are hereditary in nature. In this review, we summarize the current knowledge regarding the genetic, biochemical, and structural features of clinically relevant red blood cell enzymopathies involved in the Embden-Meyerhof pathway and the Rapoport-Luebering shunt.

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Red‐blood‐cell manufacturing methods and storage solutions differentially induce pulmonary cell activation

Vox Sanguinis ◽

10.1111/vox.12911 ◽

2020 ◽

Vol 115 (5) ◽

pp. 395-404 ◽

Cited By ~ 1

Author(s):

Mathijs R. Wirtz ◽

Ruqayyah J. Almizraq ◽

Nina C. Weber ◽

Philip J. Norris ◽

Suchitra Pandey ◽

...

Keyword(s):

Blood Cell ◽

Red Blood Cell ◽

Cell Activation ◽

Cell Manufacturing ◽

Storage Solutions ◽

Manufacturing Methods ◽

And Storage

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Red blood cell (RBC) age at collection and storage influences RBC membrane-associated carbohydrates and lectin binding

Transfusion ◽

10.1111/j.1537-2995.2007.01230.x ◽

2007 ◽

Vol 47 (6) ◽

pp. 966-968 ◽

Cited By ~ 22

Author(s):

Rosemary L. Sparrow ◽

Margaret F. Veale ◽

Geraldine Healey ◽

Katherine A. Payne

Keyword(s):

Blood Cell ◽

Red Blood Cell ◽

Lectin Binding ◽

Rbc Membrane ◽

And Storage

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Processing methods and storage duration impact extracellular vesicle counts in red blood cell units

Blood Advances ◽

10.1182/bloodadvances.2020001658 ◽

2020 ◽

Vol 4 (21) ◽

pp. 5527-5539

Author(s):

Clementine Gamonet ◽

Maxime Desmarets ◽

Guillaume Mourey ◽

Sabeha Biichle ◽

Sophie Aupet ◽

...

Keyword(s):

Blood Cell ◽

Red Blood Cell ◽

Annexin V ◽

Analysis Of Covariance ◽

Active Components ◽

Storage Duration ◽

Processing Methods ◽

Controllable Factors ◽

Filter Type ◽

And Storage

Abstract Extracellular vesicles (EVs) are active components of red blood cell (RBC) concentrates and may be associated with beneficial and adverse effects of transfusion. Elucidating controllable factors associated with EV release in RBC products is thus important to better manage the quality and properties of RBC units. Erythrocyte-derived EVs (EEVs) and platelet-derived EVs (PEVs) were counted in 1226 RBC units (administered to 280 patients) using a standardized cytometry-based method. EV size and CD47 and annexin V expression were also measured. The effects of donor characteristics, processing methods, and storage duration on EV counts were analyzed by using standard comparison tests, and analysis of covariance was used to determine factors independently associated with EV counts. PEV as well as EEV counts were higher in whole-blood–filtered RBC units compared with RBC-filtered units; PEV counts were associated with filter type (higher with filters associated with higher residual platelets), and CD47 expression was higher on EEVs in RBC units stored longer. Multivariate analysis showed that EEV counts were strongly associated with filter type (P < .0001), preparation, and storage time (+25.4 EEV/µL per day [P = .01] and +42.4 EEV/µL per day [P < .0001], respectively). The only independent factor associated with PEV counts was the residual platelet count in the unit (+67.1 PEV/µL; P < .0001). Overall, processing methods have an impact on EV counts and characteristics, leading to large variations in EV quantities transfused into patients. RBC unit processing methods might be standardized to control the EV content of RBC units if any impacts on patient outcomes can be confirmed. The IMIB (Impact of Microparticles in Blood) study is ancillary to the French ABLE (Age of Transfused Blood in Critically Ill Adults) trial (ISRCTN44878718).

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Hemoglobin FM-Fort Ripley: Another Lesson From the Neonate

PEDIATRICS ◽

10.1542/peds.83.5.792 ◽

1989 ◽

Vol 83 (5) ◽

pp. 792-793

Author(s):

BERTIL E. GLADER

Keyword(s):

Blood Cell ◽

Red Blood Cell ◽

Pediatric Patients ◽

Cytochrome B5 ◽

Fetal Hemoglobin ◽

Cytochrome B5 Reductase ◽

Aniline Derivatives ◽

Total Hemoglobin ◽

Erythrocyte Enzyme

In this issue of Pediatrics, Priest and co-workers have described a new fetal hemoglobin abnormality (Hb FM-Fort Ripley) which causes neonatal methemoglobinemia. Methemoglobin is the form of hemoglobin in which iron is oxidized (Fe3+) instead of reduced (Fe2+), and in this state hemoglobin does not transport oxygen. Methemoglobin normally accounts for less than 1% of the total hemoglobin, although small amounts of hemoglobin continually are being oxidized by exogenous and endogenous agents (including oxygen itself). Elevated methemoglobin levels occasionally occur in pediatric patients, and when this happens they are due to one or more of the following causes: exposure to chemicals that oxidize hemoglobin iron (eg, nitrates, nitrites, aniline derivatives); hereditary deficiency of methemoglobin reductase (also known as cytochrome b5 reductase), an erythrocyte enzyme that normally reduces methemoglobin; transient red blood cell deficiency of methemoglobin reductase, a normal neonatal event that persists until 3 to 4 months of age; the inheritance of an M hemoglobin, such as Hb FM-Fort Ripley.

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Heterogeneity of blood processing and storage additives in different centers impacts stored red blood cell metabolism as much as storage time: lessons from REDS‐III—Omics

Transfusion ◽

10.1111/trf.14979 ◽

2018 ◽

Vol 59 (1) ◽

pp. 89-100 ◽

Cited By ~ 25

Author(s):

Angelo D'Alessandro ◽

Rachel Culp‐Hill ◽

Julie A. Reisz ◽

Mikayla Anderson ◽

Xiaoyun Fu ◽

...

Keyword(s):

Blood Cell ◽

Red Blood Cell ◽

Storage Time ◽

Cell Metabolism ◽

Blood Processing ◽

Processing And Storage ◽

And Storage

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An Efficient Apparatus for Rapid Deoxygenation of Erythrocyte Concentrates for Alternative Banking Strategies

Journal of Blood Transfusion ◽

10.1155/2013/896537 ◽

2013 ◽

Vol 2013 ◽

pp. 1-8 ◽

Cited By ~ 6

Author(s):

Lello Zolla ◽

Angelo D'Alessandro

Keyword(s):

Blood Cell ◽

Red Blood Cell ◽

Small Scale ◽

Prolonged Storage ◽

Case Scenario ◽

Electron Microscopic ◽

Production Chain ◽

Erythrocyte Concentrates ◽

Blood Cell Morphology ◽

And Storage

Erythrocyte concentrates (ECs) stored for transfusion purposes still represent a lifesaving solution in a wide series of clinically occurring circumstances, especially for traumatized and perioperative patients. However, concerns still arise and persist as to whether current criteria for collection and storage of ECs might actually represent the best case scenario or there might rather be still room for improvement. In particular, the prolonged storage of EC has been associated with the accumulation of a wide series of storage lesions, either reversible (metabolism) or irreversible (protein and morphology). Independent laboratories have contributed to propose alternative strategies, among which is the introduction of oxygen removal treatments to ECs. Convincing biochemical and preliminary clinical evidences have been produced about the benefits derived from the introduction of this practice. We, hereby, propose a rapid, efficient, and time-effective strategy for blood deoxygenation which might fit in current EC production chain. The proposed strategy resulted in the complete deoxygenation of red blood cell hemoglobin (pO2<0.0021 mmHg). A preliminary small-scale study about the application of the present method resulted in reduced hemolysis, decreased vesiculation, and limited alterations to the red blood cell morphology, as gleaned from flow cytometry and scanning electron microscopic analyses. Further in-depth and larger-scale investigations are encouraged.

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Extracellular Vesicles from Red Blood Cell Products Induce a Strong Pro-Inflammatory Host Response, Dependent on Both Numbers and Storage Duration

Transfusion Medicine and Hemotherapy ◽

10.1159/000442681 ◽

2015 ◽

Vol 43 (4) ◽

pp. 302-305 ◽

Cited By ~ 19

Author(s):

Marleen Straat ◽

Anita N. Böing ◽

Anita Tuip-De Boer ◽

Rienk Nieuwland ◽

Nicole P. Juffermans

Keyword(s):

Blood Cell ◽

Extracellular Vesicles ◽

Red Blood Cell ◽

Host Response ◽

Storage Duration ◽

And Storage

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Chaperoning erythropoiesis

Blood ◽

10.1182/blood-2008-09-115238 ◽

2009 ◽

Vol 113 (10) ◽

pp. 2136-2144 ◽

Cited By ~ 39

Author(s):

Mitchell J. Weiss ◽

Camila O. dos Santos

Keyword(s):

Blood Cell ◽

Red Blood Cell ◽

Molecular Chaperones ◽

Protein Production ◽

Current Knowledge ◽

Cell Type ◽

Hemoglobin Synthesis ◽

Biochemical Pathways ◽

Erythroid Precursors ◽

Regulate Protein

AbstractMultisubunit complexes containing molecular chaperones regulate protein production, stability, and degradation in virtually every cell type. We are beginning to recognize how generalized and tissue-specific chaperones regulate specialized aspects of erythropoiesis. For example, chaperones intersect with erythropoietin signaling pathways to protect erythroid precursors against apoptosis. Molecular chaperones also participate in hemoglobin synthesis, both directly and indirectly. Current knowledge in these areas only scratches the surface of what is to be learned. Improved understanding of how molecular chaperones regulate erythropoietic development and hemoglobin homeostasis should identify biochemical pathways amenable to pharmacologic manipulation in a variety of red blood cell disorders including thalassemia and other anemias associated with hemoglobin instability.

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