What is coagulation disorder?

Coagulation disorder is a functional disease. When the disease occurs, the patient will show bleeding diseases and functional abnormalities. Coagulation disorder is usually divided into several stages, hereditary disease factors, and acquired factors. Hereditary coagulation disorder is a single inheritance and mostly occurs in infancy. Patients have a common family history. There is also hemophilia. Hemophilia is also a gene mutation or family genetic history, but it does not occur in everyone, and it is not ruled out that it is inherited from the previous generation. Patients with hemophilia often have organizational bleeding, muscle bleeding, and joint bleeding. They may suddenly bleed during a slight collision and friction, and the bleeding will not stop. They must go to the hospital for regular blood transfusions to control the normal balance of the body's blood. Coagulation disorders are very harmful and require timely and reasonable treatment.

Definition of terms

Coagulation diaorders refer to bleeding disorders caused by coagulation factor deficiency or dysfunction.

Clinical classification

It is divided into two categories: hereditary and acquired.

1. Hereditary coagulation disorders are generally caused by a single coagulation factor deficiency, which often causes bleeding symptoms in infancy and often has a family history.

2. Acquired coagulation dysfunction is more common. Patients often have multiple coagulation factor deficiencies. It mostly occurs in adults. Clinically, in addition to bleeding, they are also accompanied by symptoms and signs of the primary disease.

Classification Description

hemophilia

Hemophilia is the most common hereditary coagulation factor deficiency, which can be divided into two types: hemophilia A (factor VIII procoagulant component deficiency) and hemophilia B (factor IX deficiency). The biosynthetic genes of factor VIII:C and factor IX are both located on the X chromosome, so they are called X-linked diseases. Both are X-linked recessive inheritance, occurring in males and transmitted to females. Although female carriers have varying degrees of reduced factor VIII:C or factor IX activity, they generally have no bleeding symptoms. About 1/3 of patients have no family history. This may be because there are few males in the family or the disease is inherited from the previous generation and is overlooked, or it may be caused by gene mutation.

Clinical manifestations

The main symptom is bleeding, characterized by bleeding in soft tissues, muscles, and weight-bearing joints. Bleeding tendency usually occurs in early childhood, and mild cases may not be diagnosed until adolescence or even adulthood. The earlier the bleeding symptoms appear, the more serious the condition. Patients may present with severe bleeding after minor trauma or surgery, often with bleeding that does not stop during tooth extraction or minor surgery. A small number of patients experience this as the first symptom. Bleeding can continue for hours or even weeks. The degree of bleeding is related to the plasma factor activity (concentration).

Although the activity of factor VII or IX required for normal hemostasis is 25%, the factor activity in symptomatic patients is often less than 5%. Clinically, hemophilia is divided into severe, moderate, mild and subclinical types based on factor activity. The most common sites of bleeding are the vulnerable parts of the limbs. Deep tissue hematomas may occur. Large hematomas may compress nearby nerves such as the femoral nerve, median nerve, and ulnar nerve, causing pain and paralysis symptoms; compression of blood vessels may cause jaundice. Bleeding from the soft tissues of the neck and throat may cause suffocation due to airway obstruction. Retroperitoneal and mesenteric bleeding may cause abdominal pain. Severe cases may experience epistaxis, gingival bleeding, gastrointestinal bleeding, and hematuria. Excessive bleeding may cause anemia. Recurrent bleeding into the joint cavity occurs in critically ill patients, often after minor injuries, and may also occur spontaneously. There may be local swelling, pain, tenderness, and acute symptoms lasting 3 to 5 days. After the bleeding stops, the accumulated blood will be gradually absorbed after about a few weeks and no trace will be left. If it is not absorbed over time, it can cause synovitis, and repeated bleeding can cause joint stiffness, and finally lead to permanent joint damage, osteoporosis, limited joint movement, deformation, and nearby muscle atrophy, resulting in disability. The most commonly affected joints are the ankles in infants and young children and the knees in children and adults.