Related Experiment Video

Updated: Feb 27, 2026

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry
08:23

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry

Published on: November 5, 2019

10.4K

Characterization of the hypercoagulable state in patients with sickle cell disease

Nirmish Shah1, Courtney Thornburg, Marilyn J Telen

  • 1Division of Hematology/Oncology, Department of Pediatrics, Duke University, Durham, NC, USA. nirmish.shah@duke.edu

Thrombosis Research
|September 11, 2012
PubMed

Insights

Sickle cell disease (SCD) patients show higher thrombin generation and D-dimer levels during vaso-occlusive crisis (VOC) compared to steady state. These hypercoagulable markers indicate increased thrombotic risk but do not correlate with specific clinical outcomes.

Area of Science:

  • Hematology
  • Thrombosis Research
  • Vascular Biology

Background:

  • Sickle cell disease (SCD) is characterized by a complex pathophysiology.
  • A prothrombotic state is increasingly recognized as a significant factor in SCD.

Purpose of the Study:

  • To investigate thrombin generation in SCD patients using calibrated automated thrombography (CAT) and D-dimer.
  • To correlate these hypercoagulable markers with clinical disease manifestations.

Main Methods:

  • Studied 51 patients with homozygous hemoglobin S, including those in vaso-occlusive crisis (VOC) and steady state.
  • Utilized calibrated automated thrombography (CAT) and D-dimer assays.
  • Correlated findings with clinical outcomes like avascular necrosis, stroke, hospitalizations, and transcranial Doppler (TCD) velocities in pediatric patients.

Main Results:

  • Significantly higher D-dimer levels were observed during VOC compared to steady state (p<0.0001).
  • CAT analysis revealed significant differences in thrombin generation phases between crisis and steady state (p<0.0001).
  • In pediatric patients, increased TCD velocities correlated with steady state D-dimer and thrombin-antithrombin complex (p<0.05).

Conclusions:

  • Hypercoagulable markers effectively differentiate SCD patients during and between VOC.
  • These markers do not show correlation with specific clinical phenotypes in SCD patients.
Abstract

More Related Videos

Experimental and Imaging Techniques for Examining Fibrin Clot Structures in Normal and Diseased States07:09

Experimental and Imaging Techniques for Examining Fibrin Clot Structures in Normal and Diseased States

Published on: April 1, 2015

12.1K
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy07:24

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

2.0K

Related Concept Videos

Chronic Kidney Disease II: Clinical Manifestations01:24

Chronic Kidney Disease II: Clinical Manifestations

Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
768
Venous Thrombosis II: Clinical Manifestations and Diagnostic Studies01:20

Venous Thrombosis II: Clinical Manifestations and Diagnostic Studies

The key difference between Superficial Vein Thrombosis (SVT) and Deep Vein Thrombosis (DVT) lies in their location and severity.Clinical ManifestationsSVT typically presents with localized pain, tenderness, and redness along the course of a superficial vein, often accompanied by a palpable, cord-like structure under the skin. This condition is usually less dangerous than DVT but can be uncomfortable and may lead to complications such as cellulitis or, rarely, a clot extension into the deep...
391
Venous Thrombosis I: Introduction01:30

Venous Thrombosis I: Introduction

Venous thrombosis, the most common disorder of the veins, involves the formation of a thrombus or blood clot associated with vein inflammation. It can be classified as either superficial vein thrombosis or deep vein thrombosis.Superficial Vein Thrombosis: This involves the formation of a thrombus in a superficial vein, usually the greater or lesser saphenous vein. Though less severe than deep vein thrombosis (DVT), SVT can lead to complications if untreated.Deep Vein Thrombosis (DVT): This...
562
Multiple Allele Traits01:49

Multiple Allele Traits

The Concept of Multiple Allelism
38.4K
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.
2.4K
Introduction to Hemostasis01:05

Introduction to Hemostasis

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,...
15.3K