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Haemophilia & Blood Factors (from Wikipedia)
Haemophilia (/hiːməˈfɪliə/; also spelled hemophilia in North America, from the Greek haima αἷμα 'blood' and philia φιλία 'love'[1]) is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation, which is used to stop bleeding when a blood vessel is broken. Haemophilia A (clotting factor VIII deficiency) is the most common form of the disorder, present in about 1 in 5,000–10,000 male births.[2] Haemophilia B (factor IX deficiency) occurs in around 1 in about 20,000–34,000 male births.
Haemophilia lowers blood plasma clotting factor levels of the coagulation factors needed for a normal clotting process. Thus when a blood vessel is injured, a temporary scab does form, but the missing coagulation factors prevent fibrin formation, which is necessary to maintain the blood clot. A haemophiliac does not bleed more intensely than a person without it, but can bleed for a much longer time. In severe haemophiliacs even a minor injury can result in blood loss lasting days or weeks, or even never healing completely. In areas such as the brain or inside joints, this can be fatal or permanently debilitating.
Coagulation (clotting) is the process by which blood changes from a liquid to a gel. It potentially results in hemostasis, the cessation of blood loss from a damaged vessel, followed by repair. The mechanism of coagulation involves activation, adhesion, and aggregation of platelets along with deposition and maturation of fibrin. Disorders of coagulation are disease states which can result in bleeding (hemorrhage or bruising) or obstructive clotting (thrombosis).
Coagulation is highly conserved throughout biology; in all mammals, coagulation involves both a cellular (platelet) and a protein (coagulation factor) component. The system in humans has been the most extensively researched and is the best understood.
Coagulation begins almost instantly after an injury to the blood vessel has damaged the endothelium lining the vessel. Exposure of blood to the space under the endothelium initiates two processes: changes in platelets, and the exposure of subendothilial tissue factor to plasma Factor VII,which ultimately leads to fibrin formation. Platelets immediately form a plug at the site of injury; this is called primary hemostasis. Secondary hemostasis occurs simultaneously: Additional coagulation factors or clotting factors beyond Factor VII, respond in a complex cascade to form fibrin strands, which strengthen the platelet plug.
Coagulopathy (also called clotting disorder and bleeding disorder) is a condition in which the blood’s ability to clot (coagulate) is impaired. This condition can cause prolonged or excessive bleeding, which may occur spontaneously or following an injury or medical and dental procedures.
Signs and symptoms
Coagulopathy may cause uncontrolled internal or external bleeding. Left untreated, uncontrolled bleeding may cause damage to joints, muscles, or internal organs and may be life threatening. People should seek immediate medical care for serious symptoms, including heavy external bleeding, blood in the urine or stool, double vision, severe head or neck pain, repeated vomiting, difficulty walking, convulsions, or seizures. They should seek prompt medical care if they experience mild but unstoppable external bleeding or joint swelling and stiffness.
Mechanism
The normal clotting process depends on the interplay of various proteins in the blood. Coagulopathy may be caused by reduced levels or absence of blood-clotting proteins, known as clotting factors or coagulation factors. Genetic disorders, such as hemophilia and Von Willebrand's disease, can cause a reduction in clotting factors. Anticoagulants such as warfarin will also prevent clots from forming properly. Coagulopathy may also occur as a result of dysfunction or reduced levels of platelets (small disk-shaped bodies in the bloodstream that aid in the clotting process).
Treatment
If someone has coagulopathy, their health care provider may help them manage their symptoms with medications or replacement therapy. In replacement therapy, the reduced or absent clotting factors are replaced with proteins derived from human blood or created in the laboratory. This therapy may be given either to treat bleeding that has already begun or to prevent bleeding from occurring.
Critical Care
One area of treatment is managing people with major bleeding in a critical setting, like an emergency department. In these emergent situations, the common treatment is transfusing a combination of red cells with one of the following options:
The use of tranexamic acid is the only option that is currently supported by a large, randomized, controlled clinical trial, and is given to people with major bleeding after trauma. There are several possible risks to treating coagulopathies, such as transfusion-related acute lung injury, acute respiratory distress syndrome, multiple organ dysfunction syndrome, major hemorrhage, and venous thromboembolism.
THE CLOTTING FACTORS
Fourteen different blood coagulation factors are present in the body. Each plays a crucial role in the process blood clotting. Some work to increase the activity level of the other factors while some of the factors help set off the chemical cascade which leads to the development of fibrin.
Tests can be done to determine how effective your body’s ability to handle blood clotting is. These tests look at the different amounts of these blood coagulation factors that are present. When amounts are in sufficient quantities, blood clotting is normal. However, if the levels of some or all of the factors are low this could indicate a serious problem.
Each of the factors has their own levels which should be present in the body. Different deficiency levels are a cause for concern with each of the factors. For example, if one factor is 5% lower than it should be that might be a problem while another factor could be 15% lower and not pose a concern.
Reduced levels of the blood coagulation factors needed for hemostasis can be inherited through genetics or can develop thanks to other diseases, including liver disease, some forms of cancer, and even some vitamin deficiencies. When certain factors are not sufficient, they may need to be replaced through blood transfusions or with a drug known as desmopressin. These procedures are done either when bleeding is occurring or is likely to occur (prior to surgery, for example).
Factor I = Fibrinogen
Factor II = Prothrombin
Factor III = Tissue factor
Factor IV = Calcium
Factor V = Labile factor
Factor VI - Does not exist as it was named initially but later on discovered not to play a part in blood coagulation.
Factor VII = Stable factor
Factor VIII = Antihemophilic factor A
Factor IX = Antihemophilic factor B or Christmas factor (named after the first patient in whom the factor deficiency was documented)
Factor X = Stuart Prower factor
Factor XI = Antihemophilic factor C
Factor XII = Hageman factor
Factor XIII = Fibrin stabilising factor
Description of Clotting Factors
Factor Smallest Commercially Available Vial Size
Advate (Factor VIII, recombinant)* 250 units
Alphanine SD (Factor IX, human) 500 units
BeneFIX (Factor IX, recombinant) 250 units
Helixate FS (Factor VIII, recombinant) 250 units
Humate-P (Factor VIII / von Willebrand Factor Complex) 500 units VWF:RCo
Kogenate FS (Factor VIII, recombinant) 250 units
NOVOSEVEN RT (coagulation factor viia , recombinant) 1 mg
Profilnine SD (Factor IX Complex, human) 500 units
ADVATE*comes in six different dosage strengths 250 International Units (IU), 500 IU, 1000 IU, 1500 IU, 2000 IU*, 3000 IU*, 4000 IU*. The actual strength will be imprinted on the label and on the box. The six different strengths are color coded, as follows:
Dosage strength of approximately 250 International Units per vial (200 – 400 IU/vial)
Dosage strength of approximately 500 International Units per vial (401 – 800 IU/vial)
Dosage strength of approximately 1000 International Units per vial (801 – 1200 IU/vial)
Dosage strength of approximately 1500 International Units per vial (1201 – 1800 IU/vial)
Dosage strength of approximately 2000 International Units per vial (1801 – 2400 IU/vial) (*available only with 5 mL sWFI)
Dosage strength of approximately 3000 International Units per vial (2401 – 3600 IU/vial) (*available only with 5 mL sWFI)
Dosage strength of approximately 4000 International Units per vial (3601 – 4800 IU/vial) (*available only with 5 mL sWFI)
Always check the actual dosage strength printed on the label to make sure you are using the strength prescribed by your healthcare provider. Always check the expiration date printed on the box.
Blood and Blood Factors Related Websites
GROWTH FACTORS
Introduction
Growth Factor Table
Interleukin and Cytokine Table
Adipocytokine Table
Wnts, TGFs and BMPs
Discussion of Individual Factors
Epidermal Growth Factor (EGF)
Platelet-Derived Growth Factor (PDGF)
Fibroblast Growth Factors (FGFs)
Transforming Growth Factors-β TGFs-β)
Transforming Growth Factor-α (TGF-α)
Erythropoietin (Epo)
Insulin-Like Growth Factor-1 (IGF-1)
Insulin-Like Growth Factor-2 (IGF-2)
Interleukin-1 (IL-1)
Interleukin-2 (IL-2)
Interleukin-6 (IL-6)
Interleukin-8 (IL-8)
Tumor Necrosis Factor-α (TNF-α)
Tumor Necrosis Factor-β (TNF-β)
Interferon-γ (INF-γ)
Colony Stimulating Factors (CSFs)
The Clinical Use of Blood - Handbook (WHO; 2002; 222 pages)
Clotting Mechanism
OTHER RELATED LINKS
Blood Handbook by WHO
Blood Clots
Clotting Factor Replacement for Hemophilia - WebMD
Coagulation Factors
Coagulation from Wikipedia
Guidelines: Factor Products
Hemophilia Facts by CDC
How Is Hemophilia Treated?
Plasma to Recombinant Factors
The Hematologist Archive
Haemophilia & Blood Factors (from Wikipedia)
Haemophilia (/hiːməˈfɪliə/; also spelled hemophilia in North America, from the Greek haima αἷμα 'blood' and philia φιλία 'love'[1]) is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation, which is used to stop bleeding when a blood vessel is broken. Haemophilia A (clotting factor VIII deficiency) is the most common form of the disorder, present in about 1 in 5,000–10,000 male births.[2] Haemophilia B (factor IX deficiency) occurs in around 1 in about 20,000–34,000 male births.
Haemophilia lowers blood plasma clotting factor levels of the coagulation factors needed for a normal clotting process. Thus when a blood vessel is injured, a temporary scab does form, but the missing coagulation factors prevent fibrin formation, which is necessary to maintain the blood clot. A haemophiliac does not bleed more intensely than a person without it, but can bleed for a much longer time. In severe haemophiliacs even a minor injury can result in blood loss lasting days or weeks, or even never healing completely. In areas such as the brain or inside joints, this can be fatal or permanently debilitating.
- 1 Signs and symptoms
- 2 Causes
- 3 Diagnosis
- 4 Management
- 5 Epidemiology
- 6 History
- 7 See also
- 8 References
- 9 External links
Coagulation (clotting) is the process by which blood changes from a liquid to a gel. It potentially results in hemostasis, the cessation of blood loss from a damaged vessel, followed by repair. The mechanism of coagulation involves activation, adhesion, and aggregation of platelets along with deposition and maturation of fibrin. Disorders of coagulation are disease states which can result in bleeding (hemorrhage or bruising) or obstructive clotting (thrombosis).
Coagulation is highly conserved throughout biology; in all mammals, coagulation involves both a cellular (platelet) and a protein (coagulation factor) component. The system in humans has been the most extensively researched and is the best understood.
Coagulation begins almost instantly after an injury to the blood vessel has damaged the endothelium lining the vessel. Exposure of blood to the space under the endothelium initiates two processes: changes in platelets, and the exposure of subendothilial tissue factor to plasma Factor VII,which ultimately leads to fibrin formation. Platelets immediately form a plug at the site of injury; this is called primary hemostasis. Secondary hemostasis occurs simultaneously: Additional coagulation factors or clotting factors beyond Factor VII, respond in a complex cascade to form fibrin strands, which strengthen the platelet plug.
- 1 Physiology
- 2 Testing of coagulation
- 3 Role in disease
- 4 Pharmacology
- 5 Coagulation factors
- 6 History
- 7 Other species
- 8 References
- 9 Further reading
- 10 External links
Coagulopathy (also called clotting disorder and bleeding disorder) is a condition in which the blood’s ability to clot (coagulate) is impaired. This condition can cause prolonged or excessive bleeding, which may occur spontaneously or following an injury or medical and dental procedures.
Signs and symptoms
Coagulopathy may cause uncontrolled internal or external bleeding. Left untreated, uncontrolled bleeding may cause damage to joints, muscles, or internal organs and may be life threatening. People should seek immediate medical care for serious symptoms, including heavy external bleeding, blood in the urine or stool, double vision, severe head or neck pain, repeated vomiting, difficulty walking, convulsions, or seizures. They should seek prompt medical care if they experience mild but unstoppable external bleeding or joint swelling and stiffness.
Mechanism
The normal clotting process depends on the interplay of various proteins in the blood. Coagulopathy may be caused by reduced levels or absence of blood-clotting proteins, known as clotting factors or coagulation factors. Genetic disorders, such as hemophilia and Von Willebrand's disease, can cause a reduction in clotting factors. Anticoagulants such as warfarin will also prevent clots from forming properly. Coagulopathy may also occur as a result of dysfunction or reduced levels of platelets (small disk-shaped bodies in the bloodstream that aid in the clotting process).
Treatment
If someone has coagulopathy, their health care provider may help them manage their symptoms with medications or replacement therapy. In replacement therapy, the reduced or absent clotting factors are replaced with proteins derived from human blood or created in the laboratory. This therapy may be given either to treat bleeding that has already begun or to prevent bleeding from occurring.
Critical Care
One area of treatment is managing people with major bleeding in a critical setting, like an emergency department. In these emergent situations, the common treatment is transfusing a combination of red cells with one of the following options:
- Blood plasma
- Prothrombin complex concentrate, factor XIII, and fibrinogen
- Fibrinogen with tranexamic acid
The use of tranexamic acid is the only option that is currently supported by a large, randomized, controlled clinical trial, and is given to people with major bleeding after trauma. There are several possible risks to treating coagulopathies, such as transfusion-related acute lung injury, acute respiratory distress syndrome, multiple organ dysfunction syndrome, major hemorrhage, and venous thromboembolism.
THE CLOTTING FACTORS
Fourteen different blood coagulation factors are present in the body. Each plays a crucial role in the process blood clotting. Some work to increase the activity level of the other factors while some of the factors help set off the chemical cascade which leads to the development of fibrin.
Tests can be done to determine how effective your body’s ability to handle blood clotting is. These tests look at the different amounts of these blood coagulation factors that are present. When amounts are in sufficient quantities, blood clotting is normal. However, if the levels of some or all of the factors are low this could indicate a serious problem.
Each of the factors has their own levels which should be present in the body. Different deficiency levels are a cause for concern with each of the factors. For example, if one factor is 5% lower than it should be that might be a problem while another factor could be 15% lower and not pose a concern.
Reduced levels of the blood coagulation factors needed for hemostasis can be inherited through genetics or can develop thanks to other diseases, including liver disease, some forms of cancer, and even some vitamin deficiencies. When certain factors are not sufficient, they may need to be replaced through blood transfusions or with a drug known as desmopressin. These procedures are done either when bleeding is occurring or is likely to occur (prior to surgery, for example).
Factor I = Fibrinogen
Factor II = Prothrombin
Factor III = Tissue factor
Factor IV = Calcium
Factor V = Labile factor
Factor VI - Does not exist as it was named initially but later on discovered not to play a part in blood coagulation.
Factor VII = Stable factor
Factor VIII = Antihemophilic factor A
Factor IX = Antihemophilic factor B or Christmas factor (named after the first patient in whom the factor deficiency was documented)
Factor X = Stuart Prower factor
Factor XI = Antihemophilic factor C
Factor XII = Hageman factor
Factor XIII = Fibrin stabilising factor
Description of Clotting Factors
Factor Smallest Commercially Available Vial Size
Advate (Factor VIII, recombinant)* 250 units
Alphanine SD (Factor IX, human) 500 units
BeneFIX (Factor IX, recombinant) 250 units
Helixate FS (Factor VIII, recombinant) 250 units
Humate-P (Factor VIII / von Willebrand Factor Complex) 500 units VWF:RCo
Kogenate FS (Factor VIII, recombinant) 250 units
NOVOSEVEN RT (coagulation factor viia , recombinant) 1 mg
Profilnine SD (Factor IX Complex, human) 500 units
ADVATE*comes in six different dosage strengths 250 International Units (IU), 500 IU, 1000 IU, 1500 IU, 2000 IU*, 3000 IU*, 4000 IU*. The actual strength will be imprinted on the label and on the box. The six different strengths are color coded, as follows:
Dosage strength of approximately 250 International Units per vial (200 – 400 IU/vial)
Dosage strength of approximately 500 International Units per vial (401 – 800 IU/vial)
Dosage strength of approximately 1000 International Units per vial (801 – 1200 IU/vial)
Dosage strength of approximately 1500 International Units per vial (1201 – 1800 IU/vial)
Dosage strength of approximately 2000 International Units per vial (1801 – 2400 IU/vial) (*available only with 5 mL sWFI)
Dosage strength of approximately 3000 International Units per vial (2401 – 3600 IU/vial) (*available only with 5 mL sWFI)
Dosage strength of approximately 4000 International Units per vial (3601 – 4800 IU/vial) (*available only with 5 mL sWFI)
Always check the actual dosage strength printed on the label to make sure you are using the strength prescribed by your healthcare provider. Always check the expiration date printed on the box.
Blood and Blood Factors Related Websites
GROWTH FACTORS
Introduction
Growth Factor Table
Interleukin and Cytokine Table
Adipocytokine Table
Wnts, TGFs and BMPs
Discussion of Individual Factors
Epidermal Growth Factor (EGF)
Platelet-Derived Growth Factor (PDGF)
Fibroblast Growth Factors (FGFs)
Transforming Growth Factors-β TGFs-β)
Transforming Growth Factor-α (TGF-α)
Erythropoietin (Epo)
Insulin-Like Growth Factor-1 (IGF-1)
Insulin-Like Growth Factor-2 (IGF-2)
Interleukin-1 (IL-1)
Interleukin-2 (IL-2)
Interleukin-6 (IL-6)
Interleukin-8 (IL-8)
Tumor Necrosis Factor-α (TNF-α)
Tumor Necrosis Factor-β (TNF-β)
Interferon-γ (INF-γ)
Colony Stimulating Factors (CSFs)
The Clinical Use of Blood - Handbook (WHO; 2002; 222 pages)
- Introduction
- The appropriate use of blood and blood products Key points
- Appropriate and inappropriate transfusion
- Blood safety Principles of clinical transfusion practice
- Replacement fluids Key points
- Intravenous replacement therapy
- Intravenous replacement fluids
- Maintenance fluids Safety Other routes of fluid administration
- Crystalloid solutions Plasma-derived (natural) colloid solutions
- Synthetic colloid solutions
- Blood products Key points
- Whole blood Blood components
- Plasma derivatives
- Clinical transfusion procedures Key points
- Getting the right blood to the right patient at the right time
- Ordering blood Red cell compatibility testing
- Collecting blood products prior to transfusion
- Storing blood products prior to transfusion
- Administering blood products
- Monitoring the transfused patient
- Adverse effects of transfusion Key points
- Acute complications of transfusion
- Delayed complications of transfusion
- Delayed complications of transfusion: transfusion-transmitted infections
- Massive or large volume blood transfusions
- Clinical decisions on transfusion Key points
- General medicine Key points Blood, oxygen and the circulation
- Anaemia Malaria HIV/AIDS
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency
- Bone marrow failure Sickle cell disease
- Thalassaemias Bleeding disorders and transfusion
- Congenital bleeding and clotting disorders
- Acquired bleeding and clotting disorders
- Obstetrics Key points
- Haematological changes in pregnancy
- Anaemia in pregnancy
- Major obstetric haemorrhage
- Haemolytic disease of the newborn (HDN)
- Paediatrics & neonatology Key points
- Pediadiatric anaemia
- Transfusion in special clinical situations
- Bleeding and clotting disorders
- Thrombocytopenia
- Neonatal transfusion
- Surgery & anaesthesia Key points
- Transfusion in elective surgery
- Preparation of the patient
- Techniques to reduce operative blood loss
- Fluid replacement and transfusion
- Replacement of other fluid losses
- Autologous blood transfusion
- Care in the postoperative period
- Acute surgery & trauma Key points
- Assessment and resuscitation Reassessment
- Definitive management
- Other causes of hypovolaemia
- Paediatric patients
- Burns Key points Immediate management
- Assessing the severity of the burn
- Fluid resuscitation
- Continuing care of burns patients
- Glossary Back cover
Clotting Mechanism
- Introduction to Blood Coagulation
- Platelet Activation and von Willebrand Factor (vWF)
- Description of Clotting Factors
- Image of the Clotting Cascade
- Kallikrein-Kinin System in Coagulation
- Intrinsic (contact activation) Clotting Cascade
- Extrinsic (tissue factor) Clotting Cascade
- Activation of Thrombin
- Regulation of Thrombin Levels
- Protein C: Control of Coagulation and Sepsis
- Activation of Fibrinogen to Fibrin
- Dissolution of Fibrin Clots
- Coagulation Tests and Interpretations
- Clinical Significances of Hemostasis: The Bleeding Disorders
- Pharmacological Intervention in Bleeding
OTHER RELATED LINKS
Blood Handbook by WHO
Blood Clots
Clotting Factor Replacement for Hemophilia - WebMD
Coagulation Factors
Coagulation from Wikipedia
Guidelines: Factor Products
Hemophilia Facts by CDC
How Is Hemophilia Treated?
Plasma to Recombinant Factors
The Hematologist Archive