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Related Concept Videos

Disorders of Hemostasis01:24

Disorders of Hemostasis

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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.
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Two factors primarily cause thromboembolic conditions.
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Extrinsic and Intrinsic Pathways of Hemostasis01:20

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Blood clotting or coagulation involves extrinsic and intrinsic pathways, which ultimately merge into the common pathway, forming a fibrin clot.
The Extrinsic Pathway
The extrinsic pathway of coagulation is typically initiated by tissue damage that exposes blood to tissue factor (TF), a protein released by the damaged tissue cells outside the blood vessels—this interaction with TF triggers biochemical reactions involving specific clotting factors. The key player here is Factor VII, which...
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Introduction to Hemostasis01:05

Introduction to Hemostasis

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Hemostasis is a complex physiological process that prevents excessive bleeding when a blood vessel is injured. It's crucial for maintaining the integrity of the circulatory system, as it ensures that our blood remains fluid while still within the vascular network and yet clots to prevent blood loss upon vessel injury.
The three phases of hemostasis involve many clotting factors present in plasma and several substances released by platelets and injured tissue cells. It is a fast, localized,...
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Anticoagulant Drugs: Low-Molecular-Weight Heparins01:30

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Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
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Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Blood Transfusion and Agglutination02:45

Blood Transfusion and Agglutination

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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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Tail Vein Transection Bleeding Model in Fully Anesthetized Hemophilia A Mice
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Acquired hemophilia.

Jaime García-Chávez1, Abraham Majluf-Cruz2

  • 1Servicio de Hematología; Instituto Mexicano del Seguro Social, Centro Médico Nacional La Raza, Hospital de Especialidades "Dr. Antonio Fraga Mouret", Ciudad de México, México.

Gaceta Medica De Mexico
|February 7, 2020
PubMed
Summary
This summary is machine-generated.

Acquired hemophilia (AH), an autoimmune disorder targeting factor VIII, often goes undiagnosed due to low physician awareness. Early suspicion and prompt treatment are crucial for managing severe bleeding and improving outcomes in this rare condition.

Keywords:
Acquired hemophiliaAutoantibodiesAutoanticuerposAutoimmunityAutoinmunidadFactor VIIIHemofiliaHemofilia adquiridaHemophilia

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Area of Science:

  • Hematology
  • Autoimmune Disorders
  • Hemostasis

Background:

  • Acquired hemophilia (AH) is a rare autoimmune disorder characterized by autoantibodies against factor VIII.
  • It often presents with severe bleeding, particularly after childbirth or trauma, and is associated with conditions like lupus, cancer, and pregnancy.
  • Low disease awareness among healthcare professionals contributes to underdiagnosis and high mortality rates, especially in Mexico.

Purpose of the Study:

  • To review current data on acquired hemophilia.
  • To expand on the diagnosis and treatment of AH.
  • To highlight the need for increased awareness and early detection.

Main Methods:

  • Literature review of recent data on acquired hemophilia.
  • Analysis of diagnostic criteria and clinical presentation.
  • Overview of current treatment strategies for hemostasis and antibody eradication.

Main Results:

  • AH affects women of childbearing age and individuals over 70 with high incidence.
  • Diagnosis relies on suspicion of severe hemorrhage and prolonged activated partial thromboplastin time.
  • Treatment involves hemostasis (recombinant activated factor VII or prothrombin complex concentrate) and antibody eradication (cyclophosphamide, prednisone, rituximab).

Conclusions:

  • Acquired hemophilia is frequently underdiagnosed, leading to significant mortality.
  • Increased physician awareness is critical for timely diagnosis and effective management.
  • Prompt diagnosis and appropriate treatment are essential for improving patient outcomes.