Alternative name for hemophilia, a type of blood disorder involving defects in blood clotting.
Diseases involving malfunction of the blood’s clotting system or other problems that result in prolonged bleeding. Bleeding may result from problems with clotting, the platelets, or fragile blood vessels. Some bleeding disorders are congenital (present at birth), and others develop during illness or are acquired as a result of medical treatment.
Within the realm of hemostasis, a complication arises in the clotting mechanism of the blood.
Bleeding disorders encompass a group of conditions characterized by unexplained bleeding or excessively prolonged and intense bleeding following an injury. These disorders occur due to abnormalities in the processes responsible for the normal cessation of bleeding, including blood coagulation, the formation of platelet plugs (the smallest type of blood cell) to seal damaged blood vessels, and the constriction of blood vessels.
Malfunctions in the coagulation system commonly result in profound bleeding within the gastrointestinal tract, muscles, and joint cavities. On the other hand, deficiencies in platelets or abnormalities in blood vessels typically lead to superficial bleeding on the skin, gums, or the lining of the intestines or urinary tract.
Typically, these disorders arise from a deficiency or abnormality in the enzymes known as coagulation factors, which play a crucial role in blood clotting. Consequently, the formation of clots becomes sluggish, and the resulting clots are fragile, failing to effectively seal blood vessels. Coagulation abnormalities can either be present from birth (congenital) or acquired later in life.
The primary congenital coagulation defects include haemophilia, Christmas disease, and von Willebrand’s disease. These disorders are characterized by the absence or insufficient presence of specific coagulation factors in the blood. Haemophilia and Christmas disease are similar conditions, but they arise from deficiencies in two distinct coagulation factors: factor VIII and factor IX, respectively. Inheritance of these disorders follows a sex-linked pattern, typically affecting only males. Von Willebrand’s disease, on the other hand, is an inherited disorder involving defects in both platelets and factor VIII. It affects both males and females at a similar rate.
Individuals affected by any of these disorders may experience symptoms such as bruising, internal bleeding, unusually heavy menstrual periods, and excessive bleeding from wounds. In severe instances, recurrent bleeding into joints, such as the knee, can also occur.
Deficiencies in blood coagulation factors can be acquired at any stage of life due to conditions such as severe liver disease, digestive system disorders that hinder the absorption of vitamin K (essential for the production of certain coagulation factors), or the usage of anticoagulant medications.
Disseminated intravascular coagulation (DIC) is a serious and intricate acquired disorder. It can occur as a consequence of an underlying infection or a malignant tumor. In DIC, there is an excessive buildup of platelets and the formation of clots within small blood vessels. The consumption of coagulation factors surpasses their replenishment rate, often leading to severe bleeding.
Blood-clotting tests are employed to investigate coagulation disorders. In cases of severe disorders, treatment involves the replacement of the deficient coagulation factor. Factors can be sourced from fresh blood or fresh frozen plasma, while genetically engineered factors may also be utilized. Paradoxically, anticoagulants are sometimes administered to suppress excessive clotting activity in disseminated intravascular coagulation (DIC), resulting in a reduction of bleeding.
Bleeding can arise when there is an insufficient number of platelets in the bloodstream, a condition known as thrombocytopenia. The primary characteristic of this disorder is the occurrence of superficial bleeding on the skin and gums, often accompanied by numerous small bruises.
In some instances, bleeding can occur even when platelet numbers are within the normal range, as they may exhibit abnormal functioning. Platelet defects can be inherited, associated with the use of specific medications (including aspirin), or arise as complications of certain bone marrow disorders such as leukemia. Additionally, platelets can be destroyed by autoimmune disorders that may be triggered by an infection or drug treatment.
Blood tests are conducted to investigate platelet defects. In situations where bleeding is caused by a deficiency of platelets, treatment options may include intravenous platelet transfusions. In certain cases, oral corticosteroid medications may also be prescribed.
In rare instances, abnormal bleeding can be attributed to either a blood vessel abnormality or scurvy, which arises from a vitamin C deficiency. Elderly individuals and patients undergoing long-term corticosteroid treatment may experience mild abnormal bruising due to a decrease in support to the smallest blood vessels in the skin. Treatment is typically unnecessary for these cases.