Bleeding Disorders-types of Bleeding Disorders

Haemophilia- Haemophilia is a hereditary blood disease characterized by greatly prolonged clotting time. The blood fails to clot and abnormal bleeding occurs. It is sex-linked hereditary trait transmitted by normal heterozygous females who carry the recessive gene. It occurs almost exclusive in males. Two forms of haemophilia are known- (a) the classical haemophilia (Haemophilia A), due to factor VII deficiency (b) Christmas disease (haemophilia B) due to factor IX deficiency. In both the conditions, prothrombin activator cannot be formed by the intrinsic mechanism.

Purpura- Purpura is a condition with various manifestations and diverse causes characterized by haemorrhages into the skin, mucous membranes, internal organs and other tissues.Haemorrhages into the skin shows red, darkening into purple, then brownish- yellow and finally disappearing in 2 to 3 weeks. Areas of discoloration do not disappear under pressure. In this disorder, spontaneous bleeding occurs from large number of Capillaries and causes small punctuates hemorrhagic spots in many areas of the body. When they occur under the skin, they produce ‘purpuric spots’. Purpura is often caused by thrombocytopenia and then the condition is called ‘thrombocytopenia pupura’. The thrombocytopenia may be secondary to such causes like bone marrow depression, idiosyncrasy to drugs.

Thrombosis- Thrombosis is the formation, development or existence of a blood clot or thrombus within the vascular system. Thrombosis is a clot formed inside blood vessels. This is formed due to slowing of blood circulation and damage of the vascular endothelium. Thrombosis is a life- saving process when it occurs during hemorrhage, but it is a life- threatening event when it occurs at any other time because the clot can occulate a vessel and stop the blood supply to an organ or a part. Intravascular thrombosis sometimes occurs in coronary and cerebral vessels, which are called coronary thrombosis and cerebral thrombosis.

Von Willebrand’s disease (vWD)- Von Willebrand’s factor has only been recently discovered vWF and the classical factor VII together remains as a complex and the whole complex is called ‘factorVII complex’. Lack of vWF causes Von Willebrand’s disease. The outstanding features of vWD are protracted bleeding time, fall of plasma vWF concentration, a partial loss of factor VII efficiency.

Disseminated Intravascular Coagulation (DIC) – In this condition, coagulation process becomes wide spread so that clotting of blood develops intravascularly in widespread areas. Patients may, therefore, suffer from effects due to wide spread thrombosis in different blood vessels. As a result of such uncontrolled intravascular clotting the procogulant factors and thrombocytes are exhausted, resulting in failure of haemostatic mechanism also. The hemorrhage features of DIC are mainly due to the exhaustion of platelets and procoagulants.The disease occurs in association with incomplete abortion, especially, those involving the lungs, brain or prostate.