User:Synonymous-solecism/Coagulopathy
Coagulopathy | |
---|---|
Other names | Bleeding disorder |
gums
Acute presentation: blood-borne infections from treatment, intracerebral hemorrhage, subarachnoid hemorrhage, multiple organ dysfunction syndrome, organ damage and death from blood loss |
Coagulopathy (also known as a bleeding disorder) is a condition where the blood's ability to
Coagulopathies are sometimes erroneously referred to as "clotting disorders"; a clotting disorder is a predisposition to clot formation (thrombus), also known as a hypercoagulable state or thrombophilia.[4]
Overview
Normally, blood clotting begins within seconds after any
Acquired
Causes of acquired coagulopathies are broad and can include
Traumatic
Coagulopathies after traumatic injuries are a potentially preventable (with early recognition and treatment) cause of fatality.[14] Up to 40% of trauma deaths are due to uncontrolled bleeding or its consequences.[15][16] In 2003, Karim Brohi, Professor of Trauma Sciences at Queen Mary University of London, introduced the term Acute Traumatic Coagulopathy (ATC),[17] establishing that coagulopathy induced by trauma results in more severe bleeding, multiple organ dysfunction syndrome and high mortality. Traumatic coagulopathies are also associated with hypothermia, acidosis (increased acidity in the blood), blood protein and clotting factor dilution, and tissue destruction.[18]
Hereditary
Hereditary coagulopathies are
Von Willebrand disease is the most common type of hereditary coagulopathy.[21]
Hemophilia
Severe disorders
Mild bleeding disorders
Mild bleeding disorders (MBD) is a term used to signify a laboratory marker of disease such as deficiencies in clotting factors or parts of the hemostatic system without significant clinical signs or a clear phenotype. These disorders may be characterised by the presence of more frequent bleeding symptoms than in the normal population, but without serious complications.[26]
Signs and symptoms
Symptoms of coagulopathies can range from mild to severe. Some of the most common ones are:
- Minor wounds which bleed for more than ten minutes[27]
- Frequent nosebleeds (more than five in a year) which last longer than ten minutes[28]
- Heavy periods (menorrhagia)
- Low iron and anemia (often found correspondingly in people with heavy periods)
- Frequent unexplained or excessive bruising
- Heavy bleeding from dental surgery, other surgery or medical procedures, or childbirth
- Spontaneous miscarriages
- In infants, umbilical cord bleeding after birth
- connective tissue disorders)
Not all coagulopathies show obvious outward signs. For instance, Von Willebrand disease type 1 often involves non-specific mild bleeding symptoms and reduced von Willebrand factor levels show low heritability, with weak risk factors for bleeding, making it difficult to classify for diagnosis.[29] However, although Von Willebrand disease is theorised to affect an equal number of men and women, more women are diagnosed than men because the bleeding associated is more easily detected in menstruation and childbirth.[30]
Another coagulopathy that is often asymptomatic and found incidentally on routine bloodwork (such as pre-surgery assessments) is Factor XII deficiency; it's frequently called benign as other clotting factors compensate for the reduction so it doesn't cause excessive bleeding.[31] However, paradoxically, it's been linked in rare cases to thromboembolic (formation of clots which block blood vessels) complications.[32]
Furthermore, over 25% of healthy controls have been found to have at least one bleeding symptom[29] and it's estimated that 26% to 45% people have a history of nose bleeds, easy bruising, or gum bleeding,[33] making it difficult to determine if this is incidental or the result of an underlying disorder.
Diagnosis
The investigation of a patient with a bleeding symptoms begins with a full history and physical examination. It is important to look for any indications of hemostatic abnormalities such as bruising or
History
A positive history of abnormal bleeding is a prerequisite for inherited coagulopathy diagnosis and should guide further investigations. When inherited coagulopathies are suspected, standardised assessments such as Bleeding Assessment Tools (BAT) can be used to quickly assess presence and severity of bleeding symptoms. The ISTH-SSC Bleeding Assessment Tool consists of fourteen categories which allows diagnosticians to assess the presence and severity of bleeding symptoms retrospectively; a high score has high sensitivity for the presence of an inherited bleeding disorder. However, it is insensitive in children with a possible inherited bleeding disorder as they may not yet have experienced the events (eg, prior surgery) asked about.
Physical examination
Scars on the elbows and knees are a feature of hemophilia A. Grey Turner's sign is discolouration (bruising) of the skin on the flanks due to bleeding, which have been found in people with coagulopathies or who have taken much anticoagulative medication.[35]
Laboratory tests
Certain laboratory blood tests can be used to assess both acquired and inherited coagulopathies. These include bleeding time, a
Condition | Prothrombin time | Partial thromboplastin time | Bleeding time | Platelet count
|
---|---|---|---|---|
Vitamin K deficiency or warfarin | Prolonged | Normal or mildly prolonged | Unaffected | Unaffected |
Disseminated intravascular coagulation | Prolonged | Prolonged | Prolonged | Decreased |
Von Willebrand disease | Unaffected | Prolonged or unaffected | Prolonged | Unaffected |
Hemophilia
|
Unaffected | Prolonged | Unaffected | Unaffected |
Aspirin | Unaffected | Unaffected | Prolonged | Unaffected |
Thrombocytopenia | Unaffected | Unaffected | Prolonged | Decreased |
Liver failure, early | Prolonged | Unaffected | Unaffected | Unaffected |
Liver failure, end-stage | Prolonged | Prolonged | Prolonged | Decreased |
Uremia | Unaffected | Unaffected | Prolonged | Unaffected |
Congenital afibrinogenemia | Prolonged | Prolonged | Prolonged | Unaffected |
Factor V deficiency | Prolonged | Prolonged | Unaffected | Unaffected |
Factor X deficiency as seen in amyloid purpura | Prolonged | Prolonged | Unaffected | Unaffected |
Glanzmann's thrombasthenia | Unaffected | Unaffected | Prolonged | Unaffected |
Bernard–Soulier syndrome | Unaffected | Unaffected | Prolonged | Decreased or unaffected |
Factor XII deficiency | Unaffected | Prolonged | Unaffected | Unaffected |
C1INH deficiency | Unaffected | Shortened | Unaffected | Unaffected |
Complications
Coagulopathies can cause uncontrolled
Chronic
Hemophilic arthropathy (joint disease) is a known complication from chronic or repeated bleeding into joints (hemarthrosis),[39][40] which is found in hemophilia A and B.
Acute
Coagulopathies heighten the risk of acute compartment syndrome (a build-up of blood increasing pressure within a fascial compartment), even without clear cause or trauma. This can cause irreversible tissue damage and cell death and is a medical emergency.[41]
Treatment
Treatment of coagulopathies varies depending on their cause, severity or whether there is any active bleeding. The primary goal is to prevent excessive or dangerous bleeding in the least invasive way, which may be with medications or replacement therapy.
Pharmacological
Medication options for the treatment of coagulopathies remain limited.
Topical agents
In cases of external bleeding or intraoperative bleeding, some
Replacement therapy
In replacement therapy, the affected clotting factors are replaced clotting factor concentrates (CFCs) derived from either human blood (plasma-derived factor concentrates) or created in the laboratory (recombinant factor concentrates). Plasma-derived concentrates are collected from many different donors who have been screened for viruses, then they're separated into components, freeze-dried, and finally tested and treated once again to kill viruses before use.
Recombinant factor concentrates are genetically engineered using DNA. This therapy may be given either to treat bleeding that has already begun (on-demand (OD) therapy) or to prevent bleeding from occurring (prophylactic therapy).[citation needed] Prophylactic administration is utilised frequently in more severe coagulopathies.
Risks
Because between 10,000-50,000 different donations have to be pooled into a single lot before precipitating out fractions with cold ethanol,
In some cases, replacement therapy can have a serious complication of developing neutralizing antibodies (inhibitors), known as alloantibodies, due to exposure to foreign proteins (rather than an individual's own proteins). These alloantibodies may be directed against clotting factors, such as hemophilia-related factor VIII or IX inhibitors, or alloantibodies against von Willebrand factor, and make it harder to treat the patient in question as the original treatment is no longer effective.
There are several other possible risks, such as transfusion-related
Critical care
An important area in the treatment of coagulopathies is managing people with major bleeding in a
Further treatment can be transfusing a combination of red cells with one of the following options:[citation needed]
- Blood plasma
- Prothrombin complex concentrate, factor XIII, and fibrinogen
- Fibrinogen with tranexamic acid
Tranexamic acid reduces bleeding by inhibiting lysine binding sites on the zymogen plasminogen, preventing its conversion to the enzyme plasmin, a serine protease that degrades blood plasma proteins like fibrin clots.[50] Thus, tranexamic acid inhibits clot breakdown, and can help control blood loss. Administration of tranexamic acid within one hour of bleeding onset in trauma significantly reduces risk of death (and between one and three hours moderately reduces risk of death) without increased thromboembolic events. When given after three hours has elapsed, there are no recorded benefits, and it may in fact be detrimental.[51]
The use of tranexamic acid is the only option supported by reliable evidence of effectiveness from a randomized controlled trial for major traumatic bleeding,[52] with the greatest benefit to patients with severe injuries and those who receive massive transfusions.[53] However, a trial of 12,000 patients showed it's ineffective and can increase side effects in severe gastrointestinal bleeding.[54] Furthermore, there isn't any evidence for the use of tranexamic acid in children and it's not indicated for isolated head injuries.[53]
The most common acquired coagulopathy disorder seen in emergency care is thrombocytopenia, which can be the result of dilution from massive blood transfusions, and in these cases it is important not to dilute the clotting factors with further transfusions without replacement therapy.
See also
- Trauma triad of death
- Hypocoagulability
- Hypercoagulability
References
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- ^ "What is a Bleeding Disorder?". National Hemophilia Foundation. Retrieved 11 April 2021.
- ^ Rodeghiero, F.; Tosetto, A.; Abshire, T.; Arnold, D.M.; Coller, B.; James, P.; Neunert, C.; Lillicrap, D. on behalf of the ISTH/SSC Joint VWF and perinatal/pediatric hemostasis subcommittees working group (2010). "ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders: Supplementary material to the official communication of the SSC" (PDF). Journal of Thrombosis and Haemostasis. 8 (9). Retrieved 12 April 2021.
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- ^ "Acquired Hemophilia". NORD (National Organization for Rare Disorders). 14 January 2020. Retrieved 11 April 2021.
- ^ "Factor XI Deficiency". NORD (National Organization for Rare Disorders). 14 January 2020. Retrieved 11 April 2021.
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- ^ ISTH Supplementary Material 2010, "Any bleeding episode caused by superficial cuts [...] or that requires frequent bandage changes is considered significant. Insignificant bleeding from wounds includes those of duration < 10 minutes and lesions that usually require stitches in normal subjects (e.g., under the chin)."
- ^ ISTH Supplementary Material 2010, "In general, epistaxis [nosebleeds] should not be considered significant when it lasts less than 10 minutes, has a frequency of < 5 episodes/year."
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- ^ Chong, Ji Y. (April 2020). "Subarachnoid Hemorrhage (SAH) - Neurologic Disorders". MSD Manual Professional Edition. Retrieved 12 April 2021.
- ^ Chong, Ji Y. (April 2020). "Intracerebral Hemorrhage - Neurologic Disorders". MSD Manual Professional Edition. Retrieved 12 April 2021.
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