Hemophilia Case Study

It binds to the fibrin of fresh clots and the resulting compound converts adjacent plasminogen into

7.b) Damaged endothelium present an increased risk of blood clotting because it exposes the underlying collagen. Platelets bind to the collagen, and the clotting process is then

During this step, the body has one primary goal; Stop the bleeding (coagulate the blood). In order to reach this goal, as series of clotting factor proteins have

The body views the platelets as a foreign body and causes a response that produces antibodies that marks the spleen to destroy and remove the platelets. The platelet count is affected by antibodies produced by individuals with ITP. The antibody produced covers the surface of the platelets making them easily destructible by macrophages. Once the macrophages destroy the platelets faster than they are produced, the number of platelets are greatly reduced causing a decrease in blood clotting.

The friable atheroma may rupture, or its surface may ulcerate, exposing its core to platelets and plasma coagulation factors which are activated and produce a thrombus or thromboembolus.

The blood clotting process known as the coagulation cascade is complex and sophisticated which involves a multi-step. The coagulation process does more durable repairs of the vessel and very essential for closing up larger wounds and maintaining the platelet plug which allows for the formation of thrombus, or clot to form. The cascade process of coagulation is chain reaction because one step leads to next events. In each step of cascade produces a new protein of enzymes or catalysts. The Coagulation cascade leads to three different pathway an intrinsic pathway or an extrinsic pathway and at the end both of which lead to a common pathway. There are 12 known clotting factors involved in three different pathway of coagulation cascade including

Efficient inhibition of the fibrin deposition and thrombus formation by plasma protease FXIIa-neutralizing antibody, 3F7, by binding specifically to the enzymatic pocket of FXIIa in an extracorporeal membrane oxygenation (ECMO) system similar to heparin but without treatment associated increase in hemorrhage signifies thromboprotective properties of 3F7. Further, inhibition of the thrombus formation without impairing the hemostasis indicates FXII as a potential target for prevention of atherothrombosis. The use of ECMO simulating the clinical settings signifies the meaningful importance of this study to use in clinics. However, the prevention of the contact-induced FXIIa formation, thrombus formation in mice and rabbits and coagulation in-vitro with 3F7 has also been documented [59,60,61] (Figure 2). FXII activation is also mediated by inorganic polymer polyphosphate (polyP), which is stored in platelets and secreted on platelet activation [36]. Reduced fibrin accumulation and attenuated thrombus formation without increased risk

Hemophilia is a rare disorder when blood doesn't clot normally because it is short on sufficient blood-clotting proteins. Deep bleeding inside the body is big concern especially in the knees, ankles and elbows. The internal bleeding caused from this can damage the organs and tissues of the body and may be life threating. When a person without Hemophilia is injured, a system of procedures happens to make the blood turn from liquid to solid to clot the wound and make the blood flow stop. Platelets, which are cells found in the blood, combine together to form a clot at the site of bleeding. The platelets hold an enzyme that causes fibrinogen to change to fibrin which is a solid substance that doesn’t liquefy. The fibrin goes to the area of injury

begins with damage of capillaries, triggering the formation of a blood clot consisting of fibrin and fibronectin. This provisional matrix fills in the lesion and allows a variety of recruited cells to migrate into the lesion. Platelets present in the blood clot release multiple chemokines, which partici- pate in the recruitment of inflammatory cells, neutrophils, and macrophages, but also in chemotaxis and recruitment of fibroblasts and endothelial cells.

Venous thrombosis is a multifactorial disease. In the majority of thrombosis patients a risk factor is not detectable. Virchow's triad refers to three primary influences for thrombus formation, endothelial injury, stasis, turbulence or abnormal blood flow, and blood hypercoagulability (Kyrle, 2009). A shift in balance between procoagulant and anticoagulant of the endothelium is responsible for thrombotic state (Kyrle, 2009). Endothelial injury is the physical loss of endothelium leading to exposure of subendothelial extra-cellular matrix, adhesion of platelets, release of tissue factor, depletion of PGI2 and plasminogen activators (Kumar, 2010). Abnormal blood flow refers to turbulence that causes endothelial injury, which is a major influence

Hemophilia A is distinguished by extreme bleeding into soft tissues of the body known as hematoma, and bleeding into joint spaces known as hemarthroses. Hemarthroses lead to severe condition known as hemarthropathy. Repeated episodes of hemarthroses are very common to this disease. The severity of hemophilia A symptoms is directly proportional to factor VIII blood level and is classified as mild, moderate and severe.

Hemophilia is a hereditary and genetic mutation blood disease that does not have the ability to form a blood clot or coagulate from a small injury. The word hemophilia comes from two Greek words: haima - meaning blood and philia meaning to love. In order for the blood to clot properly, the plasma proteins also called factors need to be present in the blood. When the body forms antibodies to the clotting factors in the blood, it will stop the clotting factors from working. There are 13 types of clotting factors and they involve platelets to help the blood coagulate. Platelets also known as thrombocytes are small blood cells that form in your bone marrow to prevent blood loss by initiating a blood clot.

Hemophilia is an inherited bleeding disorder that is passed from mother to son. This affliction is passed in such a way due to the fact that the gene that is responsible for hemophilia is carried on the X chromosome that the child receives from his mother. For someone to be afflicted by hemophilia means that their blood has a difficult time clotting. There people do not bleed any faster than the average person, they simply cannot stop bleeding once they start bleeding. As of this current moment, there are no known cures for hemophilia, but there are many treatments that are highly effective in helping to treat many of the issues associated with the bleeding disorder. (citation)

If a person experiences levels below 50% they will not be able to form a healthy blood clot. People with Mild hemophilia A are only able to form 6% to 49% of the FVIII needed to produce a blood clot. Mild hemophilia results in people experiencing bleeding only after a serious injury or a surgery that results in bleeding. Usually, mild hemophilia will not be diagnosed until an injury or procedure involving prolonged bleeding occurs. In women, mild hemophilia can formulate heavier menstrual cycles and can also create hemorrhages after childbirth. People with moderate hemophilia A are only able to produce 1% to 5% FVIII in their blood. Patients with this tend to experience bleeding after serious or moderate injuries, surgeries, or they can have spontaneous bleeding episodes. Patients with severe hemophilia A are only able to produce 1% or less of the FVIII needed to be considered healthy. Any experiences following an injury will experience bleeding and will frequently experience bleeding, often into their joints and

This type differs from haemophilia A and B as there is no bleeding into joints and muscles. Another difference is that unlike factor VIII and IX deficiencies in the other Hemophilia types, factor XI Ievels n the blood don’t predominately correlate with its bleeding severity in the patient. “What is characteristic of factor XI deficiency is that you can have a severe factor XI deficiency with a level of less than 1% and be asymptomatic,” says Ann Hurlet-Jensen, MD, associate professor, pediatric hematology-oncology, Mount Sinai School of Medicine in New York. “On the other hand, you can have a level in the 30% to 40% range and have bleeding.” Although it is most often that those with the Haemophilia disorder display little and varied visible symptoms making early diagnosis and treatment difficult. It is also of autosomal recessive inheritance in which two recessive afflicted alleles are needed to produce afflicted offspring, thus affecting males and females with equal