Everything you need to know about bleeding disorders
Bleeding disorders are some of the most common disorders affecting almost all the people.
These are a group of disorders characterized by haemostasis and abnormal bleeding. The bleeding may be spontaneous in the form of small haemorrhages into the skin and mucous membranes. This could be related to platelet abnormalities.
An example is the purpura (small purple coloured spots that appear on the skin, mucous membranes and other organs) which occurs when small blood vessels burst, causing blood to pool under the skin.
Their causes are,
- Due to vascular abnormalities
- Due to platelet abnormalities
- Disorder of coagulation factor
- Disseminated intravascular coagulation
Haemorrhagic diseases due to vascular diseases:
- Hereditary haemorrhagic telangiectasia
- Ehlers-Danlos syndrome
- Osteogenesis imperfecta
- Marfan’s syndrome
- Meningococcal infections
- Henoch-Schonlein purpura
- Senile purpura
- Easy bruising
Haemorrhagic diseases due to platelet disorders:
Platelet bleeding disorders are due to:
- Reduction in number of platelets
- Rise in platelet count
- Defective platelet functions
Platelet disorders are suspected in people who show skin and mucosal haemorrhages and who have a longer bleeding time but a normal platelet count.
It is a disorder characterized by reduction in the platelet count. Normally the number below 100000µ/l is considered to constitute thrombocytopenia. Thrombocytopenia can occur due to impaired platelet production, accelerated platelet destruction, splenic sequestration and dilutional loss.
It is a condition in which there is an excess number of platelets than the body can handle it. The range above 400000µ/l is generally considered as thrombocytosis. It can occur following massive haemorrhage, iron deficiency, severe sepsis, marked inflammation, hemolysis or following splenectomy.
- Vitamin K deficiency
- Liver disease
- Circulating anticoagulants
- Disseminated Intravascular Coagulation (DIC)
The coagulation factors are listed below:
Haemophilia is a group of hereditary genetic disorders that affect the body’s ability to control blood clotting or coagulation, which is used to stop bleeding when a blood vessel is broken. There is a lack of a partial or essential clotting factor. It is a lifelong disorder.
Some common disorders are Haemophilia A (clotting factor VIII deficiency) which occurs in 1 in 5000 – 10000 male births and Haemophilia B (clotting factor IX deficiency) which occurs in 1 in about 20000 – 34000n male births.
It is a sex-linked recessive, X-chromosome disorder, which means it is more likely to occur in males.
History of haemophilia:
Haemophilia was abundant in the royal families of Britain, Spain, Russia and Germany in the 19th and the 20th centuries. Queen Victoria (1837 – 1907) is believed to be a carrier of haemophilia B and that she passed it on to 3 of her 9 children. Her son Leopold died of haemorrhage after a fall at the age of 30.
Types of haemophilia:
- Haemophilia A – a person with this type of haemophilia has very low levels or no factor VIII 80% of people with haemophilia have this type. It usually occurs in males. It occurs as a genetic mutation very rarely.
- Haemophilia B – a person with this disorder has very low levels or no factor IX. 20% of all people with haemophilia have this disease. It was called “Christmas disease”. About 30% of the occurrence is due to genetic mutation and it occurs in 1 in 25000 male births.
- Haemophilia C – a person with this disease has very low or is missing the factor XI. This disease can show up differently both in males and females.
Severity of haemophilia:
- Severe – about 80% of haemophilia sufferers are considered to be severe. There is prolonged bleeding after injuries and surgeries. There is prolonged bleeding in the muscles and joint bleeding can cause conditions like arthritis down the road.
- Moderate – about 10% of people with haemophilia are considered to be moderate. They experience prolonged bleeding and spontaneous bleeding epdisodes.
- Mild – about 10% of haemophilia sufferers are considered mild. They have prolonged bleeding after a serious injury or trauma. In many cases, this is not discovered until adulthood or after a long bleeding episode after an injury.
Bleeding in the mouth after a cut, frequent nosebleeds, heavy bleeding from a minor cut, bleeding that resumes after being stopped for some time.
- Blood in the urine and stools and easy bruising (from bleeding into the muscles).
- Bleeding in the joints: Tightness in the joint with no pain. It becomes hot to touch and swollen.
- Bleeding in the brain: Long-lasting painful headaches, stiff neck, sudden clumsiness, double vision, seizures.
It includes screening tests and clotting factor tests. Family history should be checked for unexplained deaths should be noted.
Patients with bleeding episodes are given an infusion of either fresh frozen plasma or a plasma enriched with the required clotting factor. Mostly factors VIII and IX are used. They are injected through veins and it is helpful is preventing severe blood loss and complications such as bleeding into muscles and joints.
Desmopressin is synthetic hormone which activates the body to prepare more of factor VIII.
RICE (Rice, Ice, Compression, Elevation) is a treatment done to manage joint bleeds. It reduces swelling and tissue damage when used together with clotting factor.
Disseminated Intravascular Coagulation (DIC):
This is a complex thrombo-haemorrhagic disorder (intravascular coagulation and haemorrhage) that occurs as secondary complications in some diseases. It is also called consumption coagulopathy or defibrination syndrome.
Its causes are,
- Massive tissue injury – in obstetrical situations
- Infections – viral infections, malaria, aspergillosis
- Widespread endothelial damage – aortic aneurysm, severe burns, acute glomerulonephritis
- Miscellaneous – snake bite, shock, heat stroke.
Thus the bleeding disorders were seen in detail.
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