Related Experiment Video
Updated: May 9, 2026
08:01
The Nijmegen Hemostasis Assay: Simultaneous Fluorogenic Measurement of Thrombin and Plasmin Generation in a Single Well
Published on: February 27, 2026
Occurrence of thrombosis in rare bleeding disorders
1National Haemophilia Center, Banco Municipal de Sangre, Caracas, Venezuela. arletteruizsaez@gmail.com
Seminars in Thrombosis and Hemostasis
|August 10, 2013
Summary
Rare bleeding disorders can paradoxically cause thrombosis. Afibrinogenemia, factor VII, and factor XI deficiencies are linked to venous or arterial clots, requiring individualized treatment considering all risk factors.
Area of Science:
- Hematology
- Thrombosis Research
- Rare Bleeding Disorders
Background:
- Some rare bleeding disorders present with a paradoxical tendency for thrombotic events.
- Afibrinogenemia, factor VII deficiency, and factor XI deficiency are frequently associated with venous or arterial thrombosis.
Purpose of the Study:
- To explore the multifactorial pathogenesis of thrombosis in rare bleeding disorders.
- To highlight the importance of individualized replacement therapy and risk factor management.
Main Methods:
- Review of reported cases and literature analysis of thrombotic events in patients with specific rare bleeding disorders.
- Examination of contributing factors including inherited/acquired thrombotic risks and specific genetic defect characteristics.
Main Results:
- Afibrinogenemia patients may experience severe thromboembolic disease.
- Congenital dysfibrinogenemia is linked to thrombosis in up to 20% of cases.
- Factor VII deficiency patients (3-4%) and Factor XI deficient patients (post-treatment) can develop thrombotic episodes.
Conclusions:
- Thrombosis in rare bleeding disorders is complex, influenced by multiple risk factors.
- Replacement therapy must be tailored, considering personal/family history and prothrombotic factors.
- Management includes controlling cardiovascular risk factors and considering prophylaxis for mild deficiencies.
Related Concept Videos
01:24Disorders 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.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
01:30Venous 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...
01:30Anticoagulant 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...
01:29Venous Thrombosis III: Interprofessional Care
Venous thrombosis requires effective prevention and treatment strategies to improve patient outcomes and reduce potential complications.Prevention StrategiesHealthcare providers must prioritize preventing venous thromboembolism (VTE) for all adult patients upon admission. Interventions depend on bleeding and thrombosis risk, medical history, current medications, diagnoses, planned procedures, and patient preferences. Patients on bed rest should change positions every two hours and, if not...
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...
01:20Extrinsic 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 forms a...
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 forms a...