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Hypercoagulation and Bleeding Disorders: Definitions, Causes, Diagnosis and Treatments.

Blood Coagulation can be simply defined as the process at which blood forms clot.
When there is  a vascular injury, the platelets clump together and stick to the edges of the damaged vessel.

They  release substances into the blood that perform a variety of functions:



1.Constricting the blood vessels to reduce bleeding,
2. Attracting more platelets to the area to enlarge the platelet plug, and
3. Initiating the work of plasma-based clotting factors, such as fibrinogen.

Through a complex mechanism involving many steps and many clotting factors, the plasma protein fibrinogen is transformed into long, sticky threads of fibrin. 

Together, the platelets and the fibrin create an intertwined meshwork that forms a stable clot. 

Consequently, fibrinolysis (lyses of the fibrin) takes place ensuring the flow of blood through the  healed vessel.

The Whole process from vasoconstriction to Platelets aggregation with the  initiation of blood coagulation factors to the process of fibrinolysis is referred to as Haemostasis.

BLEEDING DISORDERS

Bleeding  disorder is a clinical condition that results in a prolonged and rapid bleeding  due to the failure of the coagulation system.

CAUSES OF BLEEDING DISORDERS 

Protein (Coagulants) defects in the plasma (liquid portion of blood) responsible for blood clots.
-    Hereditary Causes-  Passed from Parent to Child, E.g  Haemophilia A & B caused by the deficiency or lack of the clotting proteins. Von Willebrand’s disease – deficiency of this factor  that helps platelets clump together and stick to vessel wall.

Vitamin K Deficiency: Certain Clotting factors are Vitamin. K dependent.
Liver diseases
Renal diseases (loss of clotting proteins from blood- Nephrotic Syndrome.)
Drugs (e.g quinine, aspirin) leading to thrombocytopenia  and defective platelet function.


                  SIGNS & SYMPTOMS OF BLEEDING DISORDERS

The Major sign of a bleeding disorder is a manifestation of a prolonged or excessive bleeding. 
Others  signs Include:
- Unexplained bruising
- Heavy menstrual bleeding
- Frequent Skin, Mucosal and Nose bleeds


Diagnoses of Bleeding Disorder
- Check Medical History
-   Rule out symptoms- How often do you experience bleeding, site of bleeding, what where you doing before bleeding began.
Full Blood Count 
Platelet Count  
Bleeding Time 
Prothrombin Time  (detects for deficiency of prothrombin, factors V, V11 and X)
Activated Partial Thromboplastin Time Test (screens for factors V111, IX, X, V, 11 and 1)


TREATMENT OF BLEEDING DISORDERS


Iron supplement
Blood Transfusion
Factor replacement
Fresh Frozen plasma transfusion
Cryoprecipitate administration

COMPLICATIONS OF BLEEDING DISORDER

- Cerebral bleeding
- Mesenteric bleeding
- Bleeding into the Joints
- Hepatic bleeding

HYPERCOAGULABLE DISORDER

This is a condition when blood tends to clot too much which as a result could break off and travel through the blood stream lodging in a vessel as a thrombus or an embolus .
When a blood vessel is injured, the body uses platelets & fibrin to form a blood clot (haemostasis) to prevent blood loss. If that mechanism causes too much clotting, the clot breaks free forming an embolus which travels through the circulation  and obstructs blood flow to certain organs.

Blood clots in the arteries can increase the risk for stroke, heart attack, severe leg pain. 

Venous blood clot can travel through the blood stream and cause deep vein thrombosis ( a blood clot in the veins of the pelvis, legs, arm, liver, kidney or intestines) and pulmonary embolus (clot in the lungs).

CAUSES OF HYPER-COAGULABLE CONDITIONS.

Hypercoagulable conditions are usually genetic or acquired. Genetic form is an inborn state while acquired conditions  are usually a result of surgery, trauma, medications or an underlying medical condition.

INHERITED HYPERCOAGULABLE CONDITIONS INCLUDE:
Factor V Leiden (Most common)
Prothrombin gene mutation
Elevated levels of fibrinogen or dysfunctional fibrinogen(dysfibrinogenemia)

ACQUIRED HYPERCOAGULABLE  CONDITIONS
These include:
- Cancer
- Medication used to treat cancer such as tamoxifen, bevacizumab, thalidomide and lenalidomide
- Recent Trauma or surgery
- Central venous catheter placement
- Heart attack, congestive heart failure, stroke, atrial fibrillation, and other illnesses that lead to decreased activity.

DIAGNOSIS OF HYPERCOAGULATION

Careful Medical history: 
- A family history of  abnormal blood clotting, abnormal blood clotting at a young age, Thrombosis in unusual locations or sites such as veins in the arms, portal, mesenteric, renal or cerebral. Idiopathic blood clots, recurring clots, a history of frequent miscarriages, stroke at a young age.

Laboratory Testing: 
- Echocardiogram (ECG) test to find out whether there is a blood clot in the heart.
- Angiography can be done to see if there’s a clot in the blood vessel.
Prothrombin Time –INTERNATIONAL NORMALIZED RATIO (PT-INR): 
This test is used to monitor patients taking oral anticoagulants such as WARFARIN (COUMADIN). The INR will help the doctor to determine how fast the blood is clotting and if the drug administered needs to be changed.

-    Activated Partial Thromboplastin Time (APTT) Measures the time it takes the blood to clot. It is used to monitor patients undergoing intravenous heparin anticoagulation therapy.

-  Fibrinogen level
- Assay of Factor V Leiden 
- Anti-thrombin activity
- Protein C activity
- Heparin antibodies (in patients who develop low platelets counts while exposed to heparin.
Antiphospholipid antibodies and lupus anticoagulants assay ( Important when evaluating someone who has had recurrent miscarriages  and venous or arterial thrombosis.


Testing helps to identify whether the patient is at risk for further clotting and helps determine an appropriate course and length of treatment to prevent future clots.  

It also helps to identify relatives who don’t currently have symptoms but may be at risk.

TREATMENT OF HYPERCOAGULABLE CONDITIONS

Anticoagulation Medications: In most cases, Treatment is  only needed when a blood clot develops in a vein or artery. Anticoagulants decrease the blood’s ability to clot  and prevent the formation of additional clots.
Anticoagulants Medications include: Warfarin (Coumadin) which comes in tablet form and taken orally.
Heparin is a liquid medication and is given either through intravenously  (IV) line or subcutaneously  (under the skin) .
Patients on Warfarin will need to have frequent blood tests especially the PT-INR, to evaluate  how well the medication is working and if it needs to be stopped, changed or continued.  

The blood tests are to ensure that blood clotting is kept at a safe and effective level.
Patients not on warfarin should have a normal INR between 0.9 and 1.3 while a patient on warfarin should have an INR range of 2.0 to 3.0, 2.5 to 3.5, or 3.0 to 4.0 depending on the reason for anticoagulation.
The result of the INR will determine the warfarin dose to be administered for patient  to keep the INR within the desired range.
This treatments are used to break up dangerous clots inside blood vessels. They are commonly used to treat an Ischemic Stroke (A clot in blood vessel in the brain).
It can also be used to treat clots in a lung artery (Pulmonary Embolism), Deep veins of the leg (Deep vein thrombosis-DVT) AND Myocardial infarction.

CONCLUSION

Regular check up should be encouraged and any sign of excessive bleeding from a small cut  or abnormal bleeding from any part of the body should be immediately reported to the health care provider for immediate attention.

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