Bleeding, both internal and external, is commonly treated in hospital settings. While the word “bleeding” is used to denote any part of the body that is losing blood, numerous potential causes of bleeding differ significantly from each other.
In newborns and fetuses, one potential cause is fetal and neonatal alloimmune thrombocytopenia (FNAIT). In this condition, maternal antibodies attack the platelets of the fetus. Platelets are blood cells that help blood clot to stop the bleeding. Without adequate platelet levels, bleeding is likely to go on for a prolonged period. If left untreated, this can result in organ damage and even death.
Comparison with other congenital bleeding disorders
Because FNAIT is driven by the lack of platelets in the body, newborns are typically infused with platelets to boost levels to normal. If this is carried out, the core driver of FNAIT has been removed and the child will no longer experience bleeding associated with FNAIT.
Read more about FNAIT testing and diagnosis
Other congenital bleeding disorders are all caused by a deficiency in one or more steps in the body’s natural process to stop bleeding. When bleeding is detected, a process known as the coagulation cascade is triggered to eventually cause the wound to stop bleeding. If any part of the coagulation process is deficient or missing, prolonged bleeding becomes likely.
Hemophilia
Hemophilia is a bleeding disorder due to a missing clotting factor. Clotting factors are integral to the coagulation cascade. There are two main forms of hemophilia: hemophilia A, which involves the absence of factor VIII, and hemophilia B, which involves the absence of factor IX.
Without these clotting factors, bleeds that typically stop in minutes may continue on for a long period of time. This includes minor bleeds, such as a paper cut. Today, hemophilia is primarily treated with factor replacement therapy, which is the introduction of the missing clotting factor into the patient. In addition, experimental therapies seek to edit the gene responsible for causing hemophilia in the first place.
Von Willebrand Disease
Von Willebrand disease is a condition in which von Willebrand factor (VWF) is deficient. VWF helps to stop bleeding by allowing platelets to adhere to the site of injury to form a clot. The absence of VWF thus makes it more difficult for a blood clot to form, resulting in prolonged bleeding.
There are a number of therapeutic solutions to this disease. The first is desmopressin, which helps stimulate the release of VWF from the body. The second is replacing the deficient VWF with VWF concentrate, thus allowing VWF levels to rise to threshold needed for clot formation. The third therapeutic strategy is to use antifibrinolytics, which can prevent the breakdown of blood clots once they have been formed.
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