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Disorders of Hemostasis

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.

Anticoagulant Drugs: Low-Molecular-Weight Heparins

Anticoagulant Drugs: Low-Molecular-Weight Heparins

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...

Extrinsic and Intrinsic Pathways of Hemostasis

Extrinsic and Intrinsic Pathways of Hemostasis

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...

Clot Retraction and Fibrinolysis

Clot Retraction and Fibrinolysis

After a fibrin clot is formed, the next step is clot retraction, a vital process facilitated by platelet contractile proteins, such as actin and myosin. These proteins pull the fibrin strands closer together and condense the clot. This action reduces the size of the clot, creating a smaller, denser structure that effectively seals off the damaged vessel. Clot retraction consolidates the clot and helps with wound healing by bringing the edges of the damaged blood vessel closer together.

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A Fibrin-Enriched and tPA-Sensitive Photothrombotic Stroke Model

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Prothrombin deficiency with recurrent subretinal hemorrhage.

Eros Qama¹, Kritika Krishnamurthy¹, Joseph J Mulvey¹

¹Department of Pathology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY, US.

Laboratory Medicine

|July 30, 2024

Summary

This summary is machine-generated.

Prothrombin deficiency, a rare bleeding disorder, was linked to recurrent subretinal hemorrhage and vision loss in a 67-year-old woman. Comprehensive coagulation testing is crucial for diagnosing ocular bleeding causes.

Keywords:

R500Q factor II ocular bleeding prothrombin Puerto Rico I prothrombin deficiency subretinal hemorrhage

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Published on: September 9, 2012

Area of Science:

Ophthalmology
Hematology
Genetics

Background:

Ocular hemorrhage is known in hemophilia A but not prothrombin deficiency.
Subretinal hemorrhage can cause sudden vision loss.

Purpose of the Study:

To report a case of subretinal hemorrhage in a patient with prothrombin deficiency.
To highlight the importance of coagulation workup in ocular bleeding.

Main Methods:

Case report of a 67-year-old woman with sudden vision loss.
Laboratory testing confirmed prothrombin deficiency (Factor II deficiency).
Molecular studies identified a homozygous missense mutation (G1499A/Arg500Gln).

Main Results:

The patient experienced recurrent subretinal hemorrhage, including a previous eye enucleation.
Prothrombin deficiency was confirmed, contributing to recurrent ocular bleeding.
A novel homozygous mutation R457Q was identified.

Conclusions:

Prothrombin deficiency can contribute to and worsen ocular bleeding, particularly subretinal hemorrhage.
Comprehensive coagulation screening, including Factor II activity, is vital for patients with unexplained ocular hemorrhage.
Genetic testing is recommended for isolated prothrombin level deficiencies.