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Related Concept Videos

Blood Transfusion and Agglutination02:45

Blood Transfusion and Agglutination

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.
History
The history of blood transfusion dates back to the 17th century, when early attempts were made in animals. In 1818 James Blundell, a British doctor, performed the first successful human blood transfusion. Later in 1900, Karl...
Disorders of Erythrocytes01:27

Disorders of Erythrocytes

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.
On the other...
Rh Blood Group01:19

Rh Blood Group

The Rhesus (Rh) antigen is crucial in determining blood groups and ensuring compatibility during blood transfusions.
Hemorrhagic Stroke l: Introduction01:17

Hemorrhagic Stroke l: Introduction

A hemorrhagic stroke is an acute neurological event that occurs when a weakened cerebral blood vessel ruptures, allowing blood to accumulate within or around the brain. The sudden release of blood forms a focal hematoma that increases intracranial pressure, displaces neural tissue, and can obstruct cerebrospinal fluid pathways. These effects may be compounded by intraventricular extension of the hemorrhage, cerebral edema, or compression of adjacent structures, all of which contribute to...
Erythropoiesis01:14

Erythropoiesis

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, and...
Disorders of Hemostasis01:24

Disorders of Hemostasis

Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.

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Related Experiment Video

Updated: Jun 27, 2026

Fast and Specific Assessment of the Halogenating Peroxidase Activity in Leukocyte-enriched Blood Samples
05:17

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Published on: July 28, 2016

[Paroxysmal nocturnal hemoglobinuria].

Pilar M Hernández-Campo1, Julia Almeida, Alberto Orfao

  • 1Servicio General de Citometría, Centro de Investigación del Cáncer (IBMCC-USAL/CSIC), Hospital Universitario de Salamanca, Departamento de Medicina, Universidad de Salamanca, Salamanca, España.

Medicina Clinica
|December 17, 2008
PubMed
Summary

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired blood disorder caused by PIG-A gene mutations. Diagnosis involves evaluating deficient GPI-anchored proteins, crucial for cell surface function.

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Area of Science:

  • Hematology
  • Genetics
  • Molecular Biology

Context:

  • Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic disorder.
  • It stems from somatic mutations in the PIG-A gene, impacting glycosylphosphatidylinositol (GPI) biosynthesis.
  • This leads to deficient surface expression of GPI-anchored proteins on blood cells.

Purpose:

  • To review recent advances in understanding PNH pathogenesis.
  • To discuss current diagnostic and monitoring strategies for PNH patients.

Summary:

  • PNH is characterized by PIG-A gene mutations affecting GPI anchor synthesis, resulting in a deficiency of GPI-anchored proteins.
  • Deficient expression of proteins like CD55 and CD59 increases PNH cell susceptibility to complement-mediated lysis.
  • Clinical manifestations include cytopenia, infection susceptibility, and thrombosis.

Impact:

  • Improved understanding of PNH pathogenic mechanisms.
  • Enhanced diagnostic and monitoring approaches for PNH patients.
  • Potential for better patient management and outcomes.