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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.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
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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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Coagulation01:09

Coagulation

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The coagulation phase is a critical part of the body's process to prevent blood loss following injury to blood vessels. It involves chemical reactions that form a clot to seal the injured area. The clotting process begins shortly after injury, within 15-20 seconds for severe damage and 1-2 minutes for minor injuries.
During the coagulation phase, clotting factors, or procoagulants, play a vital role in initiating and progressing the coagulation cascade. This cascade is a series of reactions...
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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.
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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.
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On the other...
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Introduction to Hemostasis01:05

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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.
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Measurement of Factor V Activity in Human Plasma Using a Microplate Coagulation Assay
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Correlation between Phenotype and Coagulation Factor Activity Level in Rare Bleeding Disorders: A Systematic Review.

Behnaz Tavasoli1, Alireza Zangooie2,3, Seyed Mehrab Safdari4

  • 1Department of Hematology and Blood Transfusion, School of Allied Medicine, Bushehr University of Medical Sciences, Bushehr, Iran.

Seminars in Thrombosis and Hemostasis
|December 5, 2024
PubMed
Summary

Rare bleeding disorders (RBDs) show varied links between coagulation factor levels and bleeding severity. A systematic review found strong correlations for fibrinogen, FX, and FXIII deficiencies, but weak or no links for FV, FVII, and FXI deficiencies.

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Area of Science:

  • Hematology
  • Genetics
  • Internal Medicine

Background:

  • Rare bleeding disorders (RBDs) constitute 3-5% of inherited bleeding conditions, often autosomal recessive.
  • Bleeding severity in RBDs is assessed using tools like the ISTH-BAT, but correlation with factor levels is inconsistent.
  • Prevalence of RBDs is higher in consanguineous populations.

Purpose of the Study:

  • To systematically review and analyze the correlation between coagulation factor activity levels and bleeding severity in patients with rare bleeding disorders.
  • To enhance understanding of the genotype-phenotype relationship in RBDs.
  • To inform improved management strategies for RBD patients.

Main Methods:

  • Systematic review following PRISMA guidelines, registered with PROSPERO (CRD42024504537).
  • PICO framework used, focusing on RBD patients.
  • Comprehensive literature search across PubMed, Scopus, and Web of Science until April 1, 2024, extracting data on bleeding severity, phenotype, and factor activity levels.

Main Results:

  • Inconsistent correlations observed between factor levels and bleeding severity across different RBDs.
  • Moderate to strong correlations found for fibrinogen, FX, and FXIII deficiencies (66%, 97%, 100% of relevant studies, respectively).
  • Weak or no correlations identified for FV, FVII, and FXI deficiencies (76%, 65%, 93% of relevant studies, respectively).

Conclusions:

  • The relationship between coagulation factor activity and bleeding severity in RBDs is complex and factor-specific.
  • Moderate to strong correlations exist for fibrinogen, FX, and FXIII deficiencies.
  • Further prospective studies using standardized bleeding assessment tools are necessary to optimize patient management.