Related Experiment Video
Updated: Jul 13, 2026
05:17
Fast and Specific Assessment of the Halogenating Peroxidase Activity in Leukocyte-enriched Blood Samples
Published on: July 28, 2016
[Paroxysmal nocturnal hemoglobinuria]
Régis Peffault de Latour1, Gérard Socié
1Service d'hématologie-greffe, hôpital Saint-Louis, 75475 Paris. gerard.socie@paris7.jussieu.fr
La Revue Du Praticien
|July 14, 2007
Summary
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare stem cell disorder. Recent research has advanced understanding of PNH pathophysiology, diagnosis, and treatment, though questions remain.
Area of Science:
- Hematology
- Stem Cell Biology
- Rare Diseases
Context:
- Paroxysmal nocturnal haemoglobinuria (PNH) is a rare clonal hematopoietic stem cell disease.
- Significant advancements in understanding PNH have occurred since the 1980s.
Purpose:
- To review the pathophysiology of PNH.
- To discuss recent diagnostic and therapeutic advances in PNH.
- To highlight unresolved questions in PNH biology and treatment.
Summary:
- PNH is characterized by a clonal expansion of hematopoietic stem cells with a deficiency in glycosylphosphatidylinositol (GPI)-anchored proteins.
- Advances include improved diagnostic techniques and the development of targeted therapies, such as complement inhibitors.
- Despite progress, the complex biology and optimal treatment strategies for PNH continue to be areas of active investigation.
Impact:
- Enhanced understanding of PNH pathophysiology.
- Improved diagnostic accuracy and earlier detection of PNH.
- Development of more effective and targeted treatments for PNH patients.
- Identification of key areas for future research in PNH biology and management.
Related Concept Videos
02:45Blood 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...
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...
01:27Disorders 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...
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...
01:19Rh Blood Group
The Rhesus (Rh) antigen is crucial in determining blood groups and ensuring compatibility during blood transfusions.
01:17Hemorrhagic 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...
01:14Erythropoiesis
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...
01:29Hemorrhagic Stroke ll: Pathophysiology
A hemorrhagic stroke develops when a cerebral blood vessel ruptures, allowing blood to escape into the surrounding brain tissue, as in intracerebral hemorrhage (ICH), or into the subarachnoid space, as in subarachnoid hemorrhage (SAH). Because the skull is a rigid compartment, the sudden presence of extravascular blood rapidly increases intracranial pressure and compresses adjacent neural structures, leading to immediate tissue injury and impaired cerebral perfusion.Mass Effect and Primary...