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Disorders of Erythrocytes01:27

Disorders of Erythrocytes

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Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
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Hypoxia01:23

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Hypoxia is a medical condition characterized by an inadequate oxygen supply to body tissues. It typically manifests as a bluish discoloration of the skin and mucosae, especially in fair-skinned individuals, when hemoglobin (Hb) saturation drops below 75%.
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Lifecycle of Erythrocytes01:22

Lifecycle of Erythrocytes

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Erythrocytes, also known as red blood cells, constantly move through blood capillaries. As a result, they damage their plasma membrane due to the continuous friction. Typically, after 100 to 120 days, erythrocytes become rigid and fragile as they wear out. As they pass through small vessels in the spleen and liver, they can get trapped and break apart into fragments.
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Erythropoiesis01:14

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Red blood cells  (RBCs) transport oxygen to all body tissues. These cells survive only for 120 days and then need to be replenished. Erythropoiesis is the process of RBC production. In healthy individuals, erythropoiesis ensures all tissues are amply supplied with oxygen. In addition, blood loss due to injury leads to a drop in the physiological oxygen level that will cause erythropoiesis. Any defect in erythropoiesis leads to several physiological disorders, including thalassemia, anemia,...
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Hemoglobin01:24

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Hemoglobin is a globular protein made up of four subunits. Two of these subunits are alpha chains, and the other two are beta chains. Each subunit contains a molecule of heme, which has an iron atom and can bind to oxygen. When an oxygen molecule binds to one heme group, it changes the shape of hemoglobin, making it easier for the other heme groups to bind oxygen as well.
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Blood Transfusion and Agglutination02:45

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Blood transfusion is a therapeutic measure to restore the blood volume after extensive blood loss due to an accident or a medical procedure. Blood transfusion involves drawing a certain amount of blood from a suitable donor and infusing it into the recipient.
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Paroxysmal nocturnal haemoglobinuria.

Anita Hill1, Amy E DeZern2,3, Taroh Kinoshita4,5

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Nature Reviews. Disease Primers
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Paroxysmal nocturnal haemoglobinuria (PNH) is a stem cell disease caused by PIGA gene mutations, leading to complement-mediated cell destruction. Eculizumab therapy effectively inhibits complement, offering a treatment for PNH patients.

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Area of Science:

  • Hematology
  • Immunology
  • Genetics

Background:

  • Paroxysmal nocturnal haemoglobinuria (PNH) is a rare clonal hematopoietic stem cell (HSC) disorder.
  • It is characterized by hemolytic anemia, thrombosis, and potential bone marrow failure.
  • PNH arises from somatic mutations in the PIGA gene within HSCs.

Purpose of the Study:

  • To explain the molecular basis of PNH.
  • To describe the clinical manifestations and pathophysiology.
  • To review current and potential therapeutic strategies for PNH.

Main Methods:

  • The study reviews the genetic basis of PNH, focusing on PIGA mutations.
  • It examines the role of glycosylphosphatidylinositol (GPI) anchor deficiency in disease pathogenesis.
  • Pathophysiological mechanisms involving complement activation and its consequences are discussed.

Main Results:

  • PIGA mutations disrupt GPI anchor synthesis, leading to deficient GPI-anchored proteins like CD55 and CD59.
  • Loss of these complement inhibitors makes PNH erythrocytes susceptible to intravascular hemolysis.
  • This hemolysis contributes significantly to PNH morbidity and mortality, including thrombosis.

Conclusions:

  • PNH pathogenesis is linked to the loss of complement regulatory proteins due to PIGA mutations.
  • Therapeutic approaches focus on inhibiting the terminal complement cascade.
  • Eculizumab, a complement inhibitor, is a highly effective and licensed treatment for PNH.