A Brief Discussion On Clotting Disorders

Disorders of clotting may be congenital or acquired.
Clotting disorder is a condition in which the blood's ability to coagulate (form clots) is impaired. This can cause a tendency toward prolonged or excessive bleeding which may occur spontaneously or following an injury or medical and dental procedures. 

hemarthroses in a patient with hemophillia

Causes: 

Congenital: 

• Hemophillia
• von Willebrand's disease. 

Acquired: 

• Vitamin K deficiency.

                - Hemorrhagic disease of the newborn

                - Obstructive jaundice

                - Fat malabsorption

• Liver disease
• Disseminated intravascular coagulation
• Autoimmune disease e.g SLE
• Massive transfusion
• Drugs

               - Heparin

               - Warfarin

               - Thrombolytic therapy. 

Clinical History: 

1. Onset and Duration: The duration of symptoms and age of presentation is very useful in determining the underlying etiology. Bleeding in the first few days of life occurs as a result of deficient vitamin K synthesis by the neonatal liver, due to immaturity. Patients with hemophillia usually present at a young age with excessive blood loss following injuries or surgical procedures , such as circumcision. Muscle hematomas and painful hemarthroses may be accompanying features. 

2. Past Medical History: Underlying medical conditions may be a reason for many of the acquired clotting disorders for example diseases of the liver like chronic hepatitis C may predispose to bleeding as the clotting factors synthesis in liver is impaired. 

Vitamin K is a fat soluble vitamin essential for the production of clotting factors II, VII, IX & X. Conditions such as obstructive jaundice, pancreatic or small bowel disease that impairs the absorption of fat will result in decreased production of the vitamin K dependent clotting factors and resulting clotting defect. 

Patients with autoimmune diseases like SLE may produce antibodies such as lupus anticoagulant which may predispose to clotting disorders.

Severe infections, malignancy, obstetric complications and burns may lead to DIC characterized by widespread consumption of clotting factors and platelets, resulting in severe coagulation disturbances. 

3. Drug History:  Coagulation abnormalities may be iatrogenic secondary to use of certain medications. Heparin and warfarin used for therapeutic anticoagulation may cause bleeding as a side effect. Disturbances in coagulation may result when more than the patient's normal circulating volume has been replaced by transfusion within 24 hrs. 

Examination: 

A general examination should be performed to identify any stigmata from hemophillia. Early onset osteoarthritis and pseudotumors may result from episodes of repeated hemorrhages. Skin necrosis and hematomas may be seen with warfarin overdose. In acquired coagulation disorders signs of liver disease may bring attention to the underlying disorder. 

Investigations: 

1. Complete Blood Count: Look for low platelet count which may be the cause for bleeding. (thrombocytopenia itself does not effect clotting times)

2. Clotting Screen: PT, aPTT, &TT are measures. 

3. Clotting factor assay can be done for congenital disorders like hemophilia. 

4. Fibrinogen and fibrin degradation products: In DIC, the platelet count and serum fibrinogen levels are low, whereas the fibrin degradation products such as D-dimers are increased.